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Congenital bilobed posterior third of the tongue: A case report
International Journal of Pediatric Otorhinolaryngology Extra (2008) 3, 140—142
www.elsevier.com/locate/ijporl
CASE REPORTS
Congenital bilobed posterior third of the tongue: A case report Mosaad Abdel-Aziz * ENT department, Faculty of Medicine, Kasr Elaini Hospital, Cairo University, Egypt Received 14 November 2007; received in revised form 31 January 2008; accepted 4 February 2008 Available online 17 March 2008
KEYWORDS Bilobed tongue; Bifid tongue; Congenital anomalies
Summary The tongue is a very important organ, as it plays an important role in deglutition and a cardinal role in articulation of speech. Bilobed or incomplete bifid tongue is not a well-known congenital anomaly for otolaryngologists. Bifid tongue involving the anterior two thirds has been reported before while no cases reported to have bifid (complete or incomplete) posterior third. A case of 5 years old girl with bilobed posterior third of her tongue is presented. According to literature search this appears to be the first case published on such lesion. # 2008 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Bilobed or incomplete bifid tongue is not a wellknown congenital anomaly for otolaryngologists. Embryologically, the tongue develops at 4 weeks from multiple origins; the anterior two thirds develop from two lateral swellings and one medial swelling, the tuberculum impar. These three swellings originate from the first branchial arch. As the lateral swellings increase in size, they overgrow the tuberculum impar and merge, forming the tongue body. The posterior one third develops from a median swelling called the copula or hypobranchial eminence that originates from the second, third, and part of the fourth pharyngeal arch. The intrinsic tongue muscles develop from myoblasts originating in occipital somites. The body of the tongue is separated from the posterior third by a V-shaped * Tel.: +20 105140161; fax: +20 25329113. E-mail address: [email protected].
groove, the terminal sulcus. In the midline of the terminal sulcus lies the foramen cecum where the thyroid gland develops [1]. Bifid tongue involving the anterior two thirds has been reported before. It may be an isolated deformity [2], or may be encountered in different syndromes such as median cleft syndrome [3], oralfacial-digital syndrome [4], or Klippel—Feil syndrome [5]. To the best of our knowledge, no cases reported to have bifid tongue (complete or incomplete) involving its posterior third. We report a case of bilobed or incomplete bifid posterior third of the tongue in a 5-year-old girl.
2. Case report A 5-year-old girl presented to the Otolaryngology Clinic of the Pediatric Hospital of Cairo University with dysarthria and hypernasality. The parents gave
1871-4048/$ — see front matter # 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2008.02.001
Bilobed posterior third of the tongue
Fig. 1
Bilobed tongue with long uvula.
history that this child was full-term baby with no family history of similar condition. Examination showed bilobed posterior one third of her tongue with long uvula entering into the cleft between both lobes (Fig. 1), the pharynx was abnormally deep and the mandible showed micrognathia. The palate was normal and the larynx was also normal in shape but abnormally displaced anterior. General medical examination showed no other congenital anomalies. CT was carried out for the child (Fig. 2); it showed a cleft in the posterior one third of the tongue that involves more than half of the lingual muscle thickness. Chromosome analysis was normal. The condition was discussed to the parents who refused any surgical interference.
3. Discussion The tongue is a very important organ, as it plays an important role in deglutition and a cardinal role in articulation of speech. Affection of this organ will lead to swallowing and speech disorders. Bifid anterior two thirds of tongue has been reported before, but bifid or bilobed posterior third has not been reported in the English literature and to our knowledge this is the first case. Most reported cases of bifid tongue described to have other congenital anomalies. Chidzonga et al. [6] described a case of bifid tongue associated with median cleft of the lower lip and mandible, Lu et al. [2] described a case of bifid tongue associated with midline cleft palate, mandible, cervical vertebrae and linea alba, Hagbigbi et al. [7] described a case of
141
Fig. 2 Coronal CT showed bilobed posterior third of tongue.
bifid tongue associated with epignathus teratoma and median glossal salivary mass, moreover many authors described this anomaly as a part of various syndromes [3—5]. A point of particular importance in our case is that no other congenital anomalies were found other than micrognathia. We expected to see a bifid epiglottis in this case as it has similar embryologic origin to the posterior third of tongue, but flexible laryngoscopy showed normal-shaped epiglottis. The posterior third of the tongue and epiglottis develop from single median swelling (the copula), so the occurrence of bifurcation of this part of the tongue is difficult to be explained especially in the presence of normal epiglottis. The copula being a single midline structure make bifurcation of the posterior third of tongue is strange and nearly impossible. Unlike the anterior two thirds of tongue that develop from two lateral swellings, defect of fusion between them will result in bifid tongue. The long uvula of the patient may be explained by deficient midline structure below it, and the abnormal anteriorly displaced larynx with deep pharynx may be explained by deficient tissue bulk anterior to them caused by the gutter between both tongue lobes.
Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at doi:10.1016/ j.pedex.2008.02.001.
142
M. Abdel-Aziz
References [1] D.T. Mueller, V.P. Callanan, Congenital malformations of the oral cavity, Otolaryngol. Clin. N. Am. 40 (2007) 141—160. [2] C.Y. Lu, R.J. Teng, J.W. Hou, T.J. Cheng, Bifid tongue associated with midline cleft palate, mandible, cervical vertebrae and linea alba, Eur. J. Pediatr. 157 (1998) 86. [3] S. Chapman, J.H. Golin, R.G. Hendel, A.D. Hockley, M.C.J. Wake, P. Weale, The median cleft face syndrome with associated cleft mandible, bifid odontoid peg and agenesis of the anterior arch of atlas, Br. J. Oral. Maxillofacial. Surg. 29 (1991) 279—281.
[4] L. Barthholdson, S.O.M. Hellstrom, O. Soderberg, A case of double tongue, Scand. J. Plast. Reconstr. Hand. Surg. 25 (1991) 93—95. [5] A.D. Widgerow, Klippel—Feil anomaly, cleft palate, and bifid tongue, Ann. Plast. Surg. 25 (1990) 216—222. [6] M.M. Chidzonga, V.M. Lopez perez, S. Mzezewa, Treatment of median cleft of the lowerlip, mandible, and bifid tongue with ankyloglossia, Int. J. Oral. Maxillofac. Surg. 25 (1996) 272—273. [7] K. Hagbigbi, M. Milles, D. Cleveland, V. Ziccardi, Epignathus teratoma with bifid tongue and median glossal salivary mass: report of a case, J. Oral. Maxillofac. Surg. 62 (2004) 379—383.
Available online at www.sciencedirect.com
www.elsevier.com/locate/ijporl
CASE REPORTS
Congenital bilobed posterior third of the tongue: A case report Mosaad Abdel-Aziz * ENT department, Faculty of Medicine, Kasr Elaini Hospital, Cairo University, Egypt Received 14 November 2007; received in revised form 31 January 2008; accepted 4 February 2008 Available online 17 March 2008
KEYWORDS Bilobed tongue; Bifid tongue; Congenital anomalies
Summary The tongue is a very important organ, as it plays an important role in deglutition and a cardinal role in articulation of speech. Bilobed or incomplete bifid tongue is not a well-known congenital anomaly for otolaryngologists. Bifid tongue involving the anterior two thirds has been reported before while no cases reported to have bifid (complete or incomplete) posterior third. A case of 5 years old girl with bilobed posterior third of her tongue is presented. According to literature search this appears to be the first case published on such lesion. # 2008 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Bilobed or incomplete bifid tongue is not a wellknown congenital anomaly for otolaryngologists. Embryologically, the tongue develops at 4 weeks from multiple origins; the anterior two thirds develop from two lateral swellings and one medial swelling, the tuberculum impar. These three swellings originate from the first branchial arch. As the lateral swellings increase in size, they overgrow the tuberculum impar and merge, forming the tongue body. The posterior one third develops from a median swelling called the copula or hypobranchial eminence that originates from the second, third, and part of the fourth pharyngeal arch. The intrinsic tongue muscles develop from myoblasts originating in occipital somites. The body of the tongue is separated from the posterior third by a V-shaped * Tel.: +20 105140161; fax: +20 25329113. E-mail address: [email protected].
groove, the terminal sulcus. In the midline of the terminal sulcus lies the foramen cecum where the thyroid gland develops [1]. Bifid tongue involving the anterior two thirds has been reported before. It may be an isolated deformity [2], or may be encountered in different syndromes such as median cleft syndrome [3], oralfacial-digital syndrome [4], or Klippel—Feil syndrome [5]. To the best of our knowledge, no cases reported to have bifid tongue (complete or incomplete) involving its posterior third. We report a case of bilobed or incomplete bifid posterior third of the tongue in a 5-year-old girl.
2. Case report A 5-year-old girl presented to the Otolaryngology Clinic of the Pediatric Hospital of Cairo University with dysarthria and hypernasality. The parents gave
1871-4048/$ — see front matter # 2008 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2008.02.001
Bilobed posterior third of the tongue
Fig. 1
Bilobed tongue with long uvula.
history that this child was full-term baby with no family history of similar condition. Examination showed bilobed posterior one third of her tongue with long uvula entering into the cleft between both lobes (Fig. 1), the pharynx was abnormally deep and the mandible showed micrognathia. The palate was normal and the larynx was also normal in shape but abnormally displaced anterior. General medical examination showed no other congenital anomalies. CT was carried out for the child (Fig. 2); it showed a cleft in the posterior one third of the tongue that involves more than half of the lingual muscle thickness. Chromosome analysis was normal. The condition was discussed to the parents who refused any surgical interference.
3. Discussion The tongue is a very important organ, as it plays an important role in deglutition and a cardinal role in articulation of speech. Affection of this organ will lead to swallowing and speech disorders. Bifid anterior two thirds of tongue has been reported before, but bifid or bilobed posterior third has not been reported in the English literature and to our knowledge this is the first case. Most reported cases of bifid tongue described to have other congenital anomalies. Chidzonga et al. [6] described a case of bifid tongue associated with median cleft of the lower lip and mandible, Lu et al. [2] described a case of bifid tongue associated with midline cleft palate, mandible, cervical vertebrae and linea alba, Hagbigbi et al. [7] described a case of
141
Fig. 2 Coronal CT showed bilobed posterior third of tongue.
bifid tongue associated with epignathus teratoma and median glossal salivary mass, moreover many authors described this anomaly as a part of various syndromes [3—5]. A point of particular importance in our case is that no other congenital anomalies were found other than micrognathia. We expected to see a bifid epiglottis in this case as it has similar embryologic origin to the posterior third of tongue, but flexible laryngoscopy showed normal-shaped epiglottis. The posterior third of the tongue and epiglottis develop from single median swelling (the copula), so the occurrence of bifurcation of this part of the tongue is difficult to be explained especially in the presence of normal epiglottis. The copula being a single midline structure make bifurcation of the posterior third of tongue is strange and nearly impossible. Unlike the anterior two thirds of tongue that develop from two lateral swellings, defect of fusion between them will result in bifid tongue. The long uvula of the patient may be explained by deficient midline structure below it, and the abnormal anteriorly displaced larynx with deep pharynx may be explained by deficient tissue bulk anterior to them caused by the gutter between both tongue lobes.
Appendix A. Supplementary data Supplementary data associated with this article can be found, in the online version, at doi:10.1016/ j.pedex.2008.02.001.
142
M. Abdel-Aziz
References [1] D.T. Mueller, V.P. Callanan, Congenital malformations of the oral cavity, Otolaryngol. Clin. N. Am. 40 (2007) 141—160. [2] C.Y. Lu, R.J. Teng, J.W. Hou, T.J. Cheng, Bifid tongue associated with midline cleft palate, mandible, cervical vertebrae and linea alba, Eur. J. Pediatr. 157 (1998) 86. [3] S. Chapman, J.H. Golin, R.G. Hendel, A.D. Hockley, M.C.J. Wake, P. Weale, The median cleft face syndrome with associated cleft mandible, bifid odontoid peg and agenesis of the anterior arch of atlas, Br. J. Oral. Maxillofacial. Surg. 29 (1991) 279—281.
[4] L. Barthholdson, S.O.M. Hellstrom, O. Soderberg, A case of double tongue, Scand. J. Plast. Reconstr. Hand. Surg. 25 (1991) 93—95. [5] A.D. Widgerow, Klippel—Feil anomaly, cleft palate, and bifid tongue, Ann. Plast. Surg. 25 (1990) 216—222. [6] M.M. Chidzonga, V.M. Lopez perez, S. Mzezewa, Treatment of median cleft of the lowerlip, mandible, and bifid tongue with ankyloglossia, Int. J. Oral. Maxillofac. Surg. 25 (1996) 272—273. [7] K. Hagbigbi, M. Milles, D. Cleveland, V. Ziccardi, Epignathus teratoma with bifid tongue and median glossal salivary mass: report of a case, J. Oral. Maxillofac. Surg. 62 (2004) 379—383.
Available online at www.sciencedirect.com