- Email: [email protected]
Congenital macroglossia: surgical and orthodontic management
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/pio
Case Report
Congenital macroglossia: surgical and orthodontic management Giampietro Farronato ∗ , Sara Salvadori, Lucia Giannini, Cinzia Maspero Fondazione Irccs Ca’ Granda, Ospedale Maggiore Policlinico, Department of Orthodontics, University of Milan
a r t i c l e
i n f o
a b s t r a c t
Article history:
Objectives: A case of congenital macroglossia is reported. The most important sign of
Received 20 April 2011
macroglossia is tongue protrusion through the lips. Tongue protrusion might influence
Accepted 1 June 2011
skeletal growth and can cause anterior open bite, proclination of upper and lower incisors and development of diastemas.
Keywords:
Materials and methods: A 4 year-old female patient was diagnosed with congenital macroglos-
Macroglossia
sia. Parents referred an abnormal tongue dimension since birth and the development of a
Maxillo-facial
progressive anterior open bite. The treatment of macroglossia included tongue reduction
Open bite
by partial glossectomy. She was seen regularly and at the age of eight years old a lingual
Orthodontics
frenectomy was performed and an orthodontic treatment was planned.
Surgical glossectomy
Results: At the end of the orthodontic treatment a Class I occlusion was obtained with correct overbite and overjet values. Conclusions: Early interception of macroglossia and surgical reduction in combination with orthodontic treatment can be seen as preventive measures to avoid the tongue influence on the development of malocclusions. © 2011 Società Italiana di Ortodonzia SIDO. Published by Elsevier Srl. All rights reserved.
1.
Introduction
The word macroglossia means large tongue and it is a congenital condition. Common causes of macroglossia are muscular hypertrophy, vascular malformations, congenital hypothyroidism, chromosomal abnormalities, such as Beckwith Wiedeman syndrome and Down syndrome. Macroglossia can be classified in two different subtypes: true macroglossia and pseudo-macroglossia in which the tongue might have a normal size but shows a displacement created by anatomic factors [1]. It is usually evident at birth and it is possible a partial and spontaneous regression with age [2].
∗
The diagnosis is usually based on subjective criteria such as tongue shape and protrusion, the presence of speech difficulties and deglutition or respiratory problems. The most important sign of macroglossia is tongue protrusion through the lips. The enlarged tongue appears clinically normal to palpation and the alveolar bone shows a reduction in thickness caused by tongue pressure. Tongue protrusion might also cause anterior open bite, proclination of upper and lower incisors and development of diastemas. Temporomandibular joint disorder and maxillofacial problems can be others clinical findings in patient with macroglossia [3]. Clinical consequences of macroglossia are noise during breathing, respiratory disorders, such as obstruction of
Corresponding author. Via Commenda 10 - 20122 Milan, Italy. E-mail address: [email protected] (G. Farronato). 1723-7785/$ – see front matter © 2011 Società Italiana di Ortodonzia SIDO. Published by Elsevier Srl. All rights reserved. doi:10.1016/j.pio.2011.06.003
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
93
superior airway, feeding difficulties leading to malnutrition and tongue infections caused by prolonged air exposition. Tongue protrusion influences skeletal growth and can lead to the development of a skeletal Class III disharmony, increased gonial angle and anterior open bite. Treatment of macroglossia is still unclear, surgical tongue reduction is recommended in order to improve aesthetic appearance, speech and dental problems [4]. Partial glossectomy often determines an improvement of the anterior open bite, although in more severe cases orthodontic treatment and orthognatic surgery might be useful for the correction of the malocclusion [5].
Figure 1 – Patient at the age of 2 years old.
Figs. 2-8 – Extraoral and intraoral frontal and lateral views at 4 years of age.
94
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
Figs. 9-11 – Intraoral views of the patients with the tongue crib.
2.
Case report
The patient was a 4-year-old female who came to the Department of Orthodontics of the University of Milan for an orthodontic visit. Parents referred an abnormal tongue dimension since birth and the development of a progressive anterior open bite (Fig. 1). The patient’s general conditions were good with an acceptable nutritional status although she presented breathing problems, especially at night.
At clinical examination the patient showed an enlarged tongue, an anterior open bite of 9 mm, diastemas, proclination of the lower incisors, and flattening of the alveolar ridge. When relaxed, the tongue protruded between the upper and lower anterior teeth (Figs. 2-8). No characteristic findings were obtained by radiography. Chromosome studies showed no alterations and congenital macroglossia was diagnosed. A surgical intervention of tongue reduction was then planned in order to assure a normal shape and function. The intervention consisted in a reduction glossectomy using a modified keyhole wedge design. This surgical technique
Figs. 12-17 – Extraoral and intraoral frontal and lateral views after the therapy with the tongue crib at 6 years of age.
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
95
was chosen because it allowed to obtain a reduction of tongue volume in three planes of space, preserving the vascular-nerve bundle. Histological study was performed on the removed tongue tissue and results showed chronic inflammatory infiltration, marked acanthosis and parakeratosic type hyperkeratosis. An important vascular proliferation at the subepithelial corion was observed. There were no histological lesions in the accessory salivary glands and at the muscular wall. The correct tongue function was then improved with speech therapy.
Fig. 18 – Intraoral view at the beginning of the functional therapy at 9 years of age.
Figs. 19-21 – Intraoral frontal and lateral view at the end of the functional therapy at 11 years of age.
Figs. 22-27 – Extraoral and intraoral frontal and lateral views at the beginning of the orthodontic fixed therapy at 12 years of age.
96
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
Figs. 28-33 – Extraoral and intraoral frontal and lateral views at the end of the orthodontic fixed therapy at 13 years of age.
Fig. 34 – Initial cephalometric radiograph at 9 years of age.
The patient was seen regularly up to age of eight years old when a lingual frenectomy was performed in order to allow good movements and to avoid an incorrect tongue posture that might lead to dental and skeletal problems such as anterior open bite and maxillary constriction. After one month a tongue crib bonded on the upper first molars was planned (Figs 9-11). This device avoids tongue interposition between the upper and lower teeth during rest position and deglutition. At the end of the treatment with the tongue crib, overjet and overbite values were within the norm. The treatment plan was then based on functional orthopaedic therapy in order to improve the neuromuscular function, favouring the advantages of skeletal growth and control teeth eruption. A Frankel III functional appliance was applied to guide normal development of basal bones during the pubertal ages (Figs. 12-18). At the end of this therapy good relationship between basal bones were observed (Figs. 1921). An upper and lower multibracket fixed appliance was then performed to align and refine the occlusion (Figs. 22-27). At the end of the orthodontic treatment a Class I occlusion was obtained with correct overbite and overjet (Figs. 28-33). Tongue shape and function were within the norm.
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
3.
97
Conclusions
Partial glossectomy, when indicated, allows the tongue to recover its normal size and morphology and has functional and aesthetic positive effects. On one side significantly improves tongue functions such as deglutition, respiration, speech and mastication. On the other side the surgical reduction avoids the negative effects that a large tongue might have on the skeletal development of the growing patient in particular considering an unfavourable mandibular growth. In this clinical case, in fact, early glossectomy in combination with orthodontic treatment can be seen as a preventive measure to avoid the tongue potential negative influence on skeletal growth and the consequent negative effect on the stability of the orthodontic results (Figs 34-36).
Conflict of interest The authors have reported no conflicts of interest.
Riassunto
Fig. 35 – Final cephalometric radiograph at 9 years of age.
Obiettivi: Il caso di una paziente con macroglossia congenita è riportato nel seguente articolo. Il segno clinico più importante della macroglossia è la protrusione della lingua tra le labbra. La protrusione linguale può influenzare la crescita scheletrica e causare morso aperto anteriore, proinclinazione degli incisivi superiori ed inferiori e sviluppo di diastemi. Materiali e metodi: A una paziente di 4 anni è stata diagnosticata la macroglossia congenita. I genitori hanno riferito un dimensione della lingua in eccesso fin dalla nascita e lo sviluppo di un progressivo morso aperto anteriore. Il trattamento della macroglossia è stato basato su una riduzione chirurgica parziale della lingua. Lei è stata monitorata costantemente ed a 8 anni è stata eseguita una frenulectomia linguale ed iniziato un trattamento ortodontico. Risultati: Alla fine del trattamento ortodontico sono stati raggiunti rapporti di prima classe molare e valori ideali di overjet ed overbite. Conclusioni: Il trattamento precoce della macroglossia tramite riduzione chirurgica parziale della lingua in combinazione con il trattamento ortodontico possono essere considerate come misure preventive per evitare l’influenza linguale e lo sviluppo della malocclusione.
Résumé
Fig. 36 – Cephalometric superimposition.
Objectifs: Présenter un cas de macroglossie congénitale. Le signe le plus important de la macroglossie porte sur la protrusion de la langue au travers des lèvres. La protrusion de la langue peut influencer la croissance du squelette et provoquer une béance, une proclinaison des incisives supérieures et inférieures et un développement de diastèmes. Matériels et méthodes: Une macroglossie congénitale a été diagnostiquée à une petite patiente, âgée de quatre ans. Ses parents faisaient état d’une dimension de la langue anormale dès sa naissance et du développement d’une béance progressive antérieure. Le traitement de la macroglossie prévoyait la réduction de la langue moyennant une glossectomie partielle. La patiente fut constamment suivie et soumise à une frénectomie linguale à l’âge de huit ans. Aussi un tractaient orthodontique fut-il planifié.
98
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
Résultats: Une classe dentaire I a été obtenue après le traitement orthodontique avec des valeurs correctes de surplomb et overbite. Conclusions: L’interception précoce de la macroglossie et la réduction chirurgicale associée d’un traitement orthodontique peuvent être interprétés comme des mesures préventives afin d’éviter l’influence de la langue sur le développement de malocclusions.
Resultados: Al final del tratamiento ortodóncico obtuvimos una clase I, con valores correctos de overbite y overjet. Conclusiones: Interceptar precozmente la macroglosia conjuntamente con la reduccion quirúrgica y el tratamiento ortodóncico pueden ser considerados como medidas preventivas para evitar la influencia de la lengua en el desarrollo de malas oclusiones.
Resumen
references
Objetivos: Presentar un caso de macroglosia congénita. El signo más importante de la macroglosia es la protrusión de la lengua a través de los labios. La protrusión de la lengua puede influir en el crecimiento del esqueleto y causar una mordida abierta anterior, proclinación de los incisivos superiores e inferiores y desarrollo de diastemas. ˜ de 4 anos ˜ Materiales y métodos: Una paciente nina con un diagnóstico de macroglosia congénita. Sus padres apuntaban una dimension anormal de la lengua desde su nacimiento y el desarollo de una mordida abierta progresiva anterior. El tratamiento de la macroglosia incluyó la reducción de lengua mediante glosectomia parcial. La paciente fue seguida con regularidad y a la edad de ocho ˜ fue sometida a una frenectomía lingual, con el planeamiento de anos un tratamiento ortodóncico.
1. Wolford LM, Cottrell DA. Diagnosis of macroglossia and indications for reduction glossectomy. Am J Orthod Dentofac Orthop 1996;110:170–7. 2. Farkas JG. Anthropometry of the head and face. 2nd ed New York: Raven; 1994, 224-333. 3. Myer CM, Hotaling AJ, Reilly JS. The diagnosis and treatment of macroglossia in children. Ear Nose Throat J 1986;65:444–8. 4. Gasparini G, Saltarel A, Carboni A, et al. Surgical management of macroglossia: discussion of 7 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;94:566–71. 5. Ruben Fernandez Garcıa de Guilarte, Berenguer Fronher B. An idiopathic case of macroglossia. J Plast Reconstr Aesthet Surg 2009;62:41–3.
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/pio
Case Report
Congenital macroglossia: surgical and orthodontic management Giampietro Farronato ∗ , Sara Salvadori, Lucia Giannini, Cinzia Maspero Fondazione Irccs Ca’ Granda, Ospedale Maggiore Policlinico, Department of Orthodontics, University of Milan
a r t i c l e
i n f o
a b s t r a c t
Article history:
Objectives: A case of congenital macroglossia is reported. The most important sign of
Received 20 April 2011
macroglossia is tongue protrusion through the lips. Tongue protrusion might influence
Accepted 1 June 2011
skeletal growth and can cause anterior open bite, proclination of upper and lower incisors and development of diastemas.
Keywords:
Materials and methods: A 4 year-old female patient was diagnosed with congenital macroglos-
Macroglossia
sia. Parents referred an abnormal tongue dimension since birth and the development of a
Maxillo-facial
progressive anterior open bite. The treatment of macroglossia included tongue reduction
Open bite
by partial glossectomy. She was seen regularly and at the age of eight years old a lingual
Orthodontics
frenectomy was performed and an orthodontic treatment was planned.
Surgical glossectomy
Results: At the end of the orthodontic treatment a Class I occlusion was obtained with correct overbite and overjet values. Conclusions: Early interception of macroglossia and surgical reduction in combination with orthodontic treatment can be seen as preventive measures to avoid the tongue influence on the development of malocclusions. © 2011 Società Italiana di Ortodonzia SIDO. Published by Elsevier Srl. All rights reserved.
1.
Introduction
The word macroglossia means large tongue and it is a congenital condition. Common causes of macroglossia are muscular hypertrophy, vascular malformations, congenital hypothyroidism, chromosomal abnormalities, such as Beckwith Wiedeman syndrome and Down syndrome. Macroglossia can be classified in two different subtypes: true macroglossia and pseudo-macroglossia in which the tongue might have a normal size but shows a displacement created by anatomic factors [1]. It is usually evident at birth and it is possible a partial and spontaneous regression with age [2].
∗
The diagnosis is usually based on subjective criteria such as tongue shape and protrusion, the presence of speech difficulties and deglutition or respiratory problems. The most important sign of macroglossia is tongue protrusion through the lips. The enlarged tongue appears clinically normal to palpation and the alveolar bone shows a reduction in thickness caused by tongue pressure. Tongue protrusion might also cause anterior open bite, proclination of upper and lower incisors and development of diastemas. Temporomandibular joint disorder and maxillofacial problems can be others clinical findings in patient with macroglossia [3]. Clinical consequences of macroglossia are noise during breathing, respiratory disorders, such as obstruction of
Corresponding author. Via Commenda 10 - 20122 Milan, Italy. E-mail address: [email protected] (G. Farronato). 1723-7785/$ – see front matter © 2011 Società Italiana di Ortodonzia SIDO. Published by Elsevier Srl. All rights reserved. doi:10.1016/j.pio.2011.06.003
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
93
superior airway, feeding difficulties leading to malnutrition and tongue infections caused by prolonged air exposition. Tongue protrusion influences skeletal growth and can lead to the development of a skeletal Class III disharmony, increased gonial angle and anterior open bite. Treatment of macroglossia is still unclear, surgical tongue reduction is recommended in order to improve aesthetic appearance, speech and dental problems [4]. Partial glossectomy often determines an improvement of the anterior open bite, although in more severe cases orthodontic treatment and orthognatic surgery might be useful for the correction of the malocclusion [5].
Figure 1 – Patient at the age of 2 years old.
Figs. 2-8 – Extraoral and intraoral frontal and lateral views at 4 years of age.
94
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
Figs. 9-11 – Intraoral views of the patients with the tongue crib.
2.
Case report
The patient was a 4-year-old female who came to the Department of Orthodontics of the University of Milan for an orthodontic visit. Parents referred an abnormal tongue dimension since birth and the development of a progressive anterior open bite (Fig. 1). The patient’s general conditions were good with an acceptable nutritional status although she presented breathing problems, especially at night.
At clinical examination the patient showed an enlarged tongue, an anterior open bite of 9 mm, diastemas, proclination of the lower incisors, and flattening of the alveolar ridge. When relaxed, the tongue protruded between the upper and lower anterior teeth (Figs. 2-8). No characteristic findings were obtained by radiography. Chromosome studies showed no alterations and congenital macroglossia was diagnosed. A surgical intervention of tongue reduction was then planned in order to assure a normal shape and function. The intervention consisted in a reduction glossectomy using a modified keyhole wedge design. This surgical technique
Figs. 12-17 – Extraoral and intraoral frontal and lateral views after the therapy with the tongue crib at 6 years of age.
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
95
was chosen because it allowed to obtain a reduction of tongue volume in three planes of space, preserving the vascular-nerve bundle. Histological study was performed on the removed tongue tissue and results showed chronic inflammatory infiltration, marked acanthosis and parakeratosic type hyperkeratosis. An important vascular proliferation at the subepithelial corion was observed. There were no histological lesions in the accessory salivary glands and at the muscular wall. The correct tongue function was then improved with speech therapy.
Fig. 18 – Intraoral view at the beginning of the functional therapy at 9 years of age.
Figs. 19-21 – Intraoral frontal and lateral view at the end of the functional therapy at 11 years of age.
Figs. 22-27 – Extraoral and intraoral frontal and lateral views at the beginning of the orthodontic fixed therapy at 12 years of age.
96
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
Figs. 28-33 – Extraoral and intraoral frontal and lateral views at the end of the orthodontic fixed therapy at 13 years of age.
Fig. 34 – Initial cephalometric radiograph at 9 years of age.
The patient was seen regularly up to age of eight years old when a lingual frenectomy was performed in order to allow good movements and to avoid an incorrect tongue posture that might lead to dental and skeletal problems such as anterior open bite and maxillary constriction. After one month a tongue crib bonded on the upper first molars was planned (Figs 9-11). This device avoids tongue interposition between the upper and lower teeth during rest position and deglutition. At the end of the treatment with the tongue crib, overjet and overbite values were within the norm. The treatment plan was then based on functional orthopaedic therapy in order to improve the neuromuscular function, favouring the advantages of skeletal growth and control teeth eruption. A Frankel III functional appliance was applied to guide normal development of basal bones during the pubertal ages (Figs. 12-18). At the end of this therapy good relationship between basal bones were observed (Figs. 1921). An upper and lower multibracket fixed appliance was then performed to align and refine the occlusion (Figs. 22-27). At the end of the orthodontic treatment a Class I occlusion was obtained with correct overbite and overjet (Figs. 28-33). Tongue shape and function were within the norm.
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
3.
97
Conclusions
Partial glossectomy, when indicated, allows the tongue to recover its normal size and morphology and has functional and aesthetic positive effects. On one side significantly improves tongue functions such as deglutition, respiration, speech and mastication. On the other side the surgical reduction avoids the negative effects that a large tongue might have on the skeletal development of the growing patient in particular considering an unfavourable mandibular growth. In this clinical case, in fact, early glossectomy in combination with orthodontic treatment can be seen as a preventive measure to avoid the tongue potential negative influence on skeletal growth and the consequent negative effect on the stability of the orthodontic results (Figs 34-36).
Conflict of interest The authors have reported no conflicts of interest.
Riassunto
Fig. 35 – Final cephalometric radiograph at 9 years of age.
Obiettivi: Il caso di una paziente con macroglossia congenita è riportato nel seguente articolo. Il segno clinico più importante della macroglossia è la protrusione della lingua tra le labbra. La protrusione linguale può influenzare la crescita scheletrica e causare morso aperto anteriore, proinclinazione degli incisivi superiori ed inferiori e sviluppo di diastemi. Materiali e metodi: A una paziente di 4 anni è stata diagnosticata la macroglossia congenita. I genitori hanno riferito un dimensione della lingua in eccesso fin dalla nascita e lo sviluppo di un progressivo morso aperto anteriore. Il trattamento della macroglossia è stato basato su una riduzione chirurgica parziale della lingua. Lei è stata monitorata costantemente ed a 8 anni è stata eseguita una frenulectomia linguale ed iniziato un trattamento ortodontico. Risultati: Alla fine del trattamento ortodontico sono stati raggiunti rapporti di prima classe molare e valori ideali di overjet ed overbite. Conclusioni: Il trattamento precoce della macroglossia tramite riduzione chirurgica parziale della lingua in combinazione con il trattamento ortodontico possono essere considerate come misure preventive per evitare l’influenza linguale e lo sviluppo della malocclusione.
Résumé
Fig. 36 – Cephalometric superimposition.
Objectifs: Présenter un cas de macroglossie congénitale. Le signe le plus important de la macroglossie porte sur la protrusion de la langue au travers des lèvres. La protrusion de la langue peut influencer la croissance du squelette et provoquer une béance, une proclinaison des incisives supérieures et inférieures et un développement de diastèmes. Matériels et méthodes: Une macroglossie congénitale a été diagnostiquée à une petite patiente, âgée de quatre ans. Ses parents faisaient état d’une dimension de la langue anormale dès sa naissance et du développement d’une béance progressive antérieure. Le traitement de la macroglossie prévoyait la réduction de la langue moyennant une glossectomie partielle. La patiente fut constamment suivie et soumise à une frénectomie linguale à l’âge de huit ans. Aussi un tractaient orthodontique fut-il planifié.
98
progress in orthodontics 1 3 ( 2 0 1 2 ) 92–98
Résultats: Une classe dentaire I a été obtenue après le traitement orthodontique avec des valeurs correctes de surplomb et overbite. Conclusions: L’interception précoce de la macroglossie et la réduction chirurgicale associée d’un traitement orthodontique peuvent être interprétés comme des mesures préventives afin d’éviter l’influence de la langue sur le développement de malocclusions.
Resultados: Al final del tratamiento ortodóncico obtuvimos una clase I, con valores correctos de overbite y overjet. Conclusiones: Interceptar precozmente la macroglosia conjuntamente con la reduccion quirúrgica y el tratamiento ortodóncico pueden ser considerados como medidas preventivas para evitar la influencia de la lengua en el desarrollo de malas oclusiones.
Resumen
references
Objetivos: Presentar un caso de macroglosia congénita. El signo más importante de la macroglosia es la protrusión de la lengua a través de los labios. La protrusión de la lengua puede influir en el crecimiento del esqueleto y causar una mordida abierta anterior, proclinación de los incisivos superiores e inferiores y desarrollo de diastemas. ˜ de 4 anos ˜ Materiales y métodos: Una paciente nina con un diagnóstico de macroglosia congénita. Sus padres apuntaban una dimension anormal de la lengua desde su nacimiento y el desarollo de una mordida abierta progresiva anterior. El tratamiento de la macroglosia incluyó la reducción de lengua mediante glosectomia parcial. La paciente fue seguida con regularidad y a la edad de ocho ˜ fue sometida a una frenectomía lingual, con el planeamiento de anos un tratamiento ortodóncico.
1. Wolford LM, Cottrell DA. Diagnosis of macroglossia and indications for reduction glossectomy. Am J Orthod Dentofac Orthop 1996;110:170–7. 2. Farkas JG. Anthropometry of the head and face. 2nd ed New York: Raven; 1994, 224-333. 3. Myer CM, Hotaling AJ, Reilly JS. The diagnosis and treatment of macroglossia in children. Ear Nose Throat J 1986;65:444–8. 4. Gasparini G, Saltarel A, Carboni A, et al. Surgical management of macroglossia: discussion of 7 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;94:566–71. 5. Ruben Fernandez Garcıa de Guilarte, Berenguer Fronher B. An idiopathic case of macroglossia. J Plast Reconstr Aesthet Surg 2009;62:41–3.