International Journal of Pediatric Otorhinolaryngology 79 (2015) 1175–1179
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Review Article
Accessory tongue: Classification and report of a case M. Hiradfar a,*, M. Bakhshaee a, R. Shojaeian a, N. Zabolinejad a, M. Forghani a, F. Mirhosseini b a b
Mashhad University of Medical Sciences, Iran Center of Pediatric Surgery, Iran
A R T I C L E I N F O
A B S T R A C T
Article history: Received 7 January 2015 Accepted 19 April 2015 Available online 11 May 2015
Background: Accessory tongue (AT) is a rare congenital anomaly. Due to rarity of AT, various terminologies are applied, including accessory tongue, bifid tongue, double tongue, cleft tongue, and supernumerary tongue. It seems that the anomaly has geographic distribution and most reported cases are from India and Middle East. No comprehensive classification has been introduced yet. So, we present a classification for AT according to review of all papers and documents that we found, and report a 2month-old male infant with this anomaly. ß 2015 Elsevier Ireland Ltd. All rights reserved.
Keywords: Accessory tongue Congenital anomaly Pediatric Cleft mandible
Contents 1. 2. 3. 4. 5.
Introduction . . . . . . . . . . . . . . . . . . . . . Report of the case and review article . Result . . . . . . . . . . . . . . . . . . . . . . . . . . Discussion . . . . . . . . . . . . . . . . . . . . . . Comment . . . . . . . . . . . . . . . . . . . . . . . Acknowledgements . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . .
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1. Introduction During the fourth week of intrauterine phase, the tongue is originated from the first three or four branchial arches and a median swelling (tuberculum impar) on the floor of pharynx and two lateral lingual swellings joining this central structure [1]. These three swelling are originated from the first pharyngeal arch [2]. Copula or hypobranchial eminence is another median swelling that is formed by mesoderm of the second, third, and part of the fourth branchial arch. It gives rise to posterior part of the tongue [2]. Sometimes, errors in the tongue development may occur which is due to anomalies in lingual tubercle [1,3]. Until yet, 37 cases were reported in literatures. Rarity of this anomaly, results in ill-defined terminology and classification for AT. Developmental abnormality
* Corresponding author. Tel.: +98 9151106209. E-mail address: [email protected] (M. Hiradfar). http://dx.doi.org/10.1016/j.ijporl.2015.04.029 0165-5876/ß 2015 Elsevier Ireland Ltd. All rights reserved.
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1175 1176 1176 1176 1178 1179 1179
of tongue may be major or minor, single or multiple [4]. So, many different kinds of AT could be happened with various degree of severity and functional disturbance. AT could be isolated (sporadic) or in association with other disorders (syndromic) such as, Opitz G BBB syndrome, Larsen syndrome, short rib syndrome, short rib polydactyly syndrome, oral–facial–digital, median cleft syndrome, Klipple–Feil anomaly, varadi syndrome, oro-maxillary, limb disorder, cleft palate, mandibular cleft, linea alba hernia, hypertelorism, scoliosis, aglossia, microglossia, macroglossia, hemi-glossia, Glossitis Rhombica Mediana, lingual thyroid, long tongue, and ankyloglossia. Sometimes, hamartoma or tumor (teratoma) may happen with AT simultaneously [3–10]. Sign and symptoms are variable in different age groups. Affected neonate may be completely asymptomatic at birth or suffered from breathing difficulties [11], choking [4] or swallowing disturbance [3]. Asymptomatic cases may be missed until childhood or following ages. Trouble in speech [3] may happen later in life in undiagnosed or untreated cases.
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M. Hiradfar et al. / International Journal of Pediatric Otorhinolaryngology 79 (2015) 1175–1179
Fig. 1. (A) Complete accessory tongue and cleft mandible. (B) OPG imaging. (C) Accessory tongue which consisted of interlacing bundles of muscular fibers and numerous serous and mucous accessory salivary glands, covered by stratified epithelium (H&E, 40). (D) Case after 5 years follow-up with good appearance and normal speech.
We report a patient with tongue duplication who was successfully treated with surgical excision and also review the previous cases to reach a novel classification. 2. Report of the case and review article A three month old female infant, a first child in a family of two, was referred to us due to failure to thrive and tongue and mandible deformity. She had a history of respiratory problem during starting feeding after birth. She hospitalized in another city due to aspiration pneumonia at one and two month old. She was delivered by cesarean section with APGAR score 8–9 and birth weight 3200 g. On admission, her weight, head circumferential, and length were 3500 g, 34 cm, and 57 cm, respectively. Her mother did not have any previous abortion, drug usage during pregnancy or diabetes induced pregnancy. In addition, no family history was found in either maternal or paternal families. In physical examination, an accessory tongue was diagnosed in left lateral side of native tongue which was slightly smaller compared to normal one (Fig. 1A). This caused the mandible to expand and deform. Also, there was evidence for double mandible bone according to OPG imaging (Fig. 1B). Lower lip had some deformity especially in mid line. At first, nasogastric tube and respiratory support were given for patient. With conservative management, breathing got better, and feeding started through nasogastric tube. Patient was discharged with good general condition after one week. Gradually, she tolerated feeding by mouth. Surgical intervention was performed in order to excise the accessory tongue with preservation of native tongue, nerves and salivary glands at age one year. Macroscopic results of accessory tongue demonstrated the resection of 4 cm 1.8 cm 1 cm accessory tongue covering by soft mucous membrane. Histopathological examination showed interlacing bundles of muscle fibers and numerous serous and mucous accessory salivary glands scattered throughout the muscle and lamina propria, covered by stratified epithelium and was consistent with normal tongue (Fig. 1C). Postoperative course was uneventful, no complication was observed and patient discharged after 4 days with good condition and ability to feed. We followed the case for 5 years and fortunately, she was growing up normally without any neurodevelopmental defect as well as with completely normal speech. Based on searching keywords (i.e., accessory tongue, cleft mandible, congenital anomaly and pediatric) through various sources like Google scholar, pubmed, and science direct, 34 articles were collected that the oldest and newest are related to 1966 and 2012. Generally, there are 38 cases that listed as following (Table 1). 3. Result According to this review, we found 37 cases with AT. 62% of cases were female. Associated anomalies were observed in 83% of
cases. The most common associated anomaly was cleft palate that was found in 43% of cases. Symptomatic and asymptomatic cases were 37% and 62%, respectively. Speech difficulty was reported in 3 cases with the prevalence of 5% and 2% in dorsal and lateral surfaces, respectively. The most prevalent associated clinical symptom was respiratory difficulties in 18% of cases. Incomplete form of AT was more dominant than complete types (55%). Complete form was mostly asymptomatic and only seen in 15%. 4. Discussion Despite of lack of genetic pattern in these cases, it seems that the prevalence of AT is more common in female gender. The exact frequency of AT is not clear in the world. Lack of proper data may be due to unavailability of good classification. Accordingly, and during our conducted surveys, we can suggest a suitable classification that helps to distinguish different aspects of this abnormality (Table 2). AT may be associated with other types of midline clefts that the most common are cleft palate and mandibular cleft. Many reported cases about congenital pathology of tongue are cystic lesions that are not related to AT. It is likely that the cysts of tongue are as a part of duplication of GIT. Given the introduction of AT as tongue duplication in some papers, it makes misunderstanding that AT can also be a subtype of GIT duplication [16–19]. Embryologically, with regard to lack of communication between construction of accessory tongue and GIT, and no reports about the simultaneity of GIT duplication in above cases, it seems that the nature of AT is completely independent and not related to GIT duplication. Because AT is placed in a group of midline defects [8], thus, monitoring the involved cases for these probable anomalies is suggested before surgical excision. The prenatal sonography is popularized now and it is possible to diagnose AT with this modality of fetus evaluation before birth [6,8]. In case of prenatal diagnosis of AT, due to probable respiratory problem just after birth, it is better to be referred mothers to more equipped departments. As polyhydramnios may be coexist with AT, prenatal sonographer must evaluate the fetus for this anomaly in these cases [9]. In postnatal period, evaluation for other midline defects is advised. We checked our case by brain MRI and OPG. No abnormality was seen in brain study despite of conducted survey by Manjila et al. [9] that shows duplication in pituitary glands. It is important to consider diabetic mothers as a predisposing factor in occurrence of AT [2,15,20]. We did not find any history of diabetes in mother of our case. In differential diagnosis, the benign lesions must be considered such as fibroma and haemangioma [1]. Until now, it is not revealed any genetic abnormalities in all the reported cases. Therefore, it seems that geographic distribution may be environmental or caused by other reasons. Rai et al. [2] described that this anomaly is more obvious in the southern coastal belt of India with the prevalence of 0.3%. Moreover, no inheritance pattern or family history has been observed.
M. Hiradfar et al. / International Journal of Pediatric Otorhinolaryngology 79 (2015) 1175–1179
1177
Table 1 Review of cases. Characteristics
Sex
Age
Sign/symptom
Localization
Complete/ incomplete
Associated anomalies
–Complete midline cleft of lower lip –Cleft palate –Dimple in the midline of the mandible –Epidermoid cyst No data
Cases Case 1
Chidzonga et al. [12]
F
Neonate
Non-symptom except for transient cyanosis
Anterior
Inc
Case 2
Comess et al. [15]
No data
No data
No data
No data
Case 3 Case 4
Widgerow [22] Chapman et al. [23]
No data F F
18-month-old –
– –
Inferolateral –
Inc –
Case 5 Case 6
Chidzonga et al. [24] Rao et al. [25]
F F
Neonate 9-h-old
– Anterior
– Inc
Case 7
Hunt and Vujanic [26]
–
Prenatal
– Aspiration pneumonia and severe respiratory distress –
–
–
Case 8
Lu et al. [7]
F
Neonate
–
Side to side
C
Case 9
Vandenhaute et al. [14]
F
–
Severe respiratory difficulties
Side to side
Inc
Cases 10 and 11
Britto et al. [11]
M
Neonate
Side to side
Inc
Case 12
Haghighi et al. [27]
F M
Neonate 1-day-old
Inferolateral Anterior
C C
Cases 13 and 14
Driva et al. [28]
F
Acute respiratory embarrassment Unable to feed adequately Respiratory difficulty and difficulty in oral feeding –
Side to side
Inc
–
Side to side
Inc
–
Inferior
Inc
–Cleft of secondary palate –Cleft palate –Cleft of the lower lip – –Midline cleft of the mandible –Micrognathia –Severe bilateral pulmonary hypoplasia –Centrally located tumor –Midline cleft of mandible and cervical vertebrae –Mild peripheral pulmonary stenosis –Linea alba hernia –Microretrognathia –Cleft palate –Intracranial tumor with extension oral cavity –U-shaped Cleft palate –Mandibular epilus –Epignathus teratoma –Cleft palate –Oral–facial–digital syndrome type 1 –Oral–facial–digital syndrome –Right mandibular and maxillary hypoplasia No associated congenital anomaly –Microretrognathia –Cleft palate –Micrognathia
Case 15
Konas et al. [29]
F
4-years3-months-old 6-years10-months-old Two-month-old
Case 16
F
18-year-old
–
Anterior
Inc
Case 17
Tewari and Shukla [3] James et al. [30]
–
10-h-old
Severe respiratory distress
Side to side
Inc
Case 18
Orhan et al. [31]
–
Prenatal
–
–
–
Cases 19 and 20
Bhattacharya et al. [4]
F
9-month-old
Choking during feeding
Anterolateral
Inc
F
19-year-old
Speech impairment
Dorsal
Inc
Dorsal
Inc
Side to side
Inc
No associated congenital defects
Dorsal
Inc
–Polydactyly –Micrognathia –Cleft palate – –Cleft lip –Bilateral cleft palate –Ankyloglossia –Van der woude syndrome –Incomplete median cleft of the lower lip –Complete median mandibular cleft –Ankyloglossia –Midline cleft of the lower lip and the mandible
F
Case 21
Kumar et al. [1]
M
28-year-old
Case 22
Surej et al. [5]
M
45-year-old
Case 23
–
6-weeks-old
Case 24
Andrade and Raikwar [32] Ryan et al. [33]
Difficulty in swallowing and speech Difficulty in speech and unusual appearance of tongue Difficulty in swallowing
M
Infant
–
Side to side
C
Case 25 Case 26
Martin et al. [34] Sarode et al. [35]
F M
1-day-old 7-year-old
– –
– –
– –
Case 27
Halli et al. [36]
F
6-month-old
–
–
Inc
Case 28
Bhattacharyya et al. [13]
M
5-month-old
–
Side to side
C
Case 29
Bonet et al. [37]
–
–
–
–
–
–Cleft palate –Lingual thyroid –Cleft palate –Lingual hamartoma –
–
M. Hiradfar et al. / International Journal of Pediatric Otorhinolaryngology 79 (2015) 1175–1179
1178 Table 1 (Continued ) Characteristics
Sex
Age
Sign/symptom
Localization
Complete/ incomplete
Associated anomalies
–Micrognathia –Cleft palate –Pierre-Robin Syndrome No associated congenital anomaly –Cleft of the lower lip involving the chin and the mandible –Inflamed median cervical cyst –Cleft palate –Scoliosis –Duplication of pituitary gland –Bilateral cleft lower lip – –Median Cleft palate –Median cleft lip – –Intraoral hamartomas –Incomplete cleft of the median upper lip cleft –Ankyloglossia –Brachydactyly –Cleft mandible –Lip deformity
Cases Case 30
Daniel et al. [6]
–
Prenatal
Breathing difficulties
Side to side
Inc
Case 31
Tewari and Shukla [3]
F
18-year-old
–
Anterior
Inc
Case 32
Anselem et al. [8]
F
Prenatal
–
Inferior
Inc
Case 33
Manjila et al. [9]
F
11-year-old
–
Side to side
C
Case 34 Case 35
Sharma and Passi [38] Panigrahi et al. [39]
F F
17-year-old 11-year-old
– –
– –
– –
Case 36 Case 37
Wang et al. [40] Gonzales et al. [41]
M F
29-year-old Neonate
– –
Anterior –
Inc Inc
Case 38
M. Hiradfar et al.
F
4.5-month-old
Respiratory and feeding difficulties
Side to side
Inc
Fig. 2. Gross anatomy and position.
In most cases, treatment includes surgical excision. However, this procedure may not relieve all of symptoms, especially in speech impairments. In addition, reduction glossoplasty is another method to treat these patients if feasible [8]. As shown in Table 1, speech disturbance was observed in three cases with the age more than 19 year old. Therefore, it is supposed that delay in surgical intervention may cause this difficulty. Early
Table 2 Suggested Classification. A: Gross anatomy I) AT similar to normal tongue, the size is the same as normal tongue (Fig. 2A) II) AT similar to normal tongue, the size is smaller than normal one (Fig. 2B) III) Any mass or tumors unlike a normal tongue that confirmed as a tongue tissue in microscopic evaluation (Fig. 2C) B: Position I) Side to side (lateral) (Fig. 2A and B) II) Over or under the native tongue (Fig. 2C and D) C: Sign/symptom I) Asymptomatic II) Symptomatic: respiratory, swallowing, and speech symptoms
intervention in our case and perfect speech at this time may also support this hypothesis. Besides the congenital form of bifid tongue, recently due to tongue piercing, acquired form of bifid tongue as a complication of this procedure may happen that is completely different in etiology symptoms and management compared to congenital form [21].
5. Comment AT is a rare anomaly with special geographic widespread. Due to lack of classification and ill-defined terminology, the exact frequency of this congenital anomaly may be obscure yet. Our classification may help better understanding of AT. According to this review, some of the cases had respiratory or feeding problems, but it seems that the severity of these difficulties were not as much as those which required emergency neonatal surgery. It is likely that early surgical intervention prevents trouble in speech at later ages. Therefore, performing an operation before starting speaking is recommended. This policy also prevents the chance of iatrogenic trauma to adjacent organs due to unnecessary operation in neonatal period.
M. Hiradfar et al. / International Journal of Pediatric Otorhinolaryngology 79 (2015) 1175–1179
Acknowledgment At the end, we are really appreciative of Mrs. Ghazaleh Azim Zadeh Khosravi for her worthy assistant in collecting patient’s data. References [1] S. Kumar, S.K. Tiwary, A.K. Khanna, An accessory tongue, Singap. Med. J. 50 (5) (2009) e172. [2] R. Rai, A.R. Rai, R. Rai, K. Bhat, B.V. Muralimanju, Prevalence of bifid tongue and ankyloglossia in South Indian population with an emphasis on its embryogenesis, Int. J. Morphol. 30 (1) (2012) 182–184. [3] M. Tewari, H.S. Shukla, A rare anomaly: an accessory tongue, Indian J. Surg. 69 (February (1)) (2007). [4] V. Bhattacharya, S. Khanna, A.S. Bashir, U. Kumar, R.S. Garbyal, Cleft palate associated with hamartomatous bifid tongue. Report of two cases, J. Plast. Reconstr. Aesthet. Surg. 62 (2009) 1442–1445. [5] K.L.K. Surej, N.M. Kurien, M.P. Sivan, Isolated congenital bifid tongue, Natl. J. Maxillofac. Surg. 1 (July–December (2)) (2010) 187–189. [6] E. Daniel-Spiegel, M. Ben-Ami, Bifid tongue, a rare congenital malformation, is a prenatal clue for secondary cleft palate, J. Ultrasound Med. 31 (2012) 505–509. [7] C.-Y. Lu, R.-J. Teng, J.-W. Hou, Bifid tongue associated with midline cleft palate, mandible, cervical vertebrae and linea alba, Eur. J. Pediatr. 157 (1998) 86. [8] A. Vincent-Rohfritsch, O. Anselem, G. Grange, C. Benard, G. Viot, P. Lalau, et al., Prenatal diagnosis of a cleft of the tongue, lower lip and mandible, Ultrasound Obstet. Gynecol. 39 (2012) 110–114. [9] S. Manjila, A.E. Miller, S. Vadera, K.R. Goel, R.F. Khan, C. Crowe, et al., Duplication of the pituitary gland associated with multiple blastogenesis defects: duplication of the pituitary gland (DPG)-plus syndrome. Case report and review of literature, Surg. Neurol. Int. 3 (2012) 23. [10] E.N. Emmanouil-Nikoloussi, C. Kerameos-Foroglou, Developmental malformation of human tongue and associated syndromes (review), Bull. Group Int. Rech. Sci. Stomatol. Odontol. 35 (March–June (1–2)) (1992) 5–12. [11] J.A. Britto, R.H. Ragoowansi, B.C. Sommerlad, Double tongue, intraoral anomalies, and cleft palate – case reports and a discussion of developmental pathology, Cleft Palate-Craniofac. J. 37 (July (4)) (2000). [12] M.M. Chidzonga, K.J. Shija, Congenital median cleft of the lower lip, bifid tongue with ankyloglossia, cleft palate, and submental epidermoid cyst: report of a case, J. Oral Maxillofac. Surg. 46 (1966) 809–812. [13] C.N. Bhattacharyya, K. Kalita, M. Gogoi, K.P. Deuri, Tessier 30 facial cleft, J. Indian Assoc. Pediatr. Surg. 17 (2) (2012) 75–77. [14] B. Vandenhaute, E. Leteurtre, M. Lecomte-houcke, P. Pellerin, J.P. Nuyts, J.M. Cuisset, et al., Epignathus teratoma: report of three cases with a review of the literature, Cleft Palate-Craniofac. J. 37 (January (1)) (2000). [15] L.J. Comess, P.H. Bennett, T.A. Burch, M. Miller, Congenital anomalies and diabetes in the Prima Indians of Arizona, Diabetes 18 (1969) 471–477. [16] E.A. Ameh, A.O. Jimoh, A.H. Rafindadi, S.M. Shehu, Sublingual gastric duplication cyst causing respiratory obstruction: case report, East Afr. Med. J. 77 (July (7)) (2000) 394–395. [17] M. Blanchard, N. Kadlub, S. Boudjemaa, S. Cassier, C. Garel, G. Audry, et al., Tongue cyst in children: foregut duplication, a possible diagnosis, Rev. Stomatol. Chir. Maxillofac. 113 (December (6)) (2012) 442–447. [18] E. Vimalakar Reddy, G. Ram Mohan, G. Raghavendra Prasad, G. Sathyanarayana, Lingual gastric duplication cyst in a new born, Indian J. Otolaryngol. Head Neck Surg. 63 (October (4)) (2011) 405–406.
1179
[19] S. Hambarde, P. Bendre, D. Taide, Foregut duplication cyst presenting as lingual swelling: case report and review of literature, Natl. J. Maxillofac. Surg. 2 (January (1)) (2011) 2–5. [20] A.W. James, K. Culver, B. Hall, M. Golabi, Bifid tongue: a rare feature associated with infants of diabetic mother syndrome, Am. Med. Genet. A 143A (2007) 2035–2039. [21] P.S. Fleming, T.R. Flood, Bifid tongue – a complication of tongue piercing, Br. Dent. J. 198 (March (5)) (2005). [22] A.D. Widgerow, Klipple–Feil anomaly, cleft palate, and bifid tongue, Ann. Plast. Surg. 25 (September (3)) (1990). [23] S. Chapman, H. Goldin, 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 angenesis of the anterior arch of atlas, Br. J. Oral Mexillofac. Surg. 29 (1991) 279–281. [24] M.M. Chidzonga, V.M. Lopez Perez, S. Mzezewa, Treatment of median cleft of the lower lip, mandible and bifid tongue with ankyloglossia, Int. J. Oral Maxillofac. Surg. 25 (1996) 272–273. [25] S. Rao, S. Oak, M. Wagh, B. Kulkarni, Congenital midline palatomandibular bony fusion with a mandibular cleft and a bifid tongue, Br. J. Plast. Surg. 50 (1997) 139–141. [26] N.C.A. Hunt, G.M. Vujanic, Fibrochondrogenesis in a 17-week fetus: a case expanding the phenotype, Am. J. Med. Genet. 75 (2015) 326–329. [27] K. Haghighi, 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. [28] T. Driva, D. Franklin, P.J.M. Crawford, Variation in expression of oral–facial–digital syndrome (type I): report of two cases, Int. J. Paediatr. Dent. 14 (2004) 61–68. [29] E. Konas, H.I. Canter, M.E. Mavili, Goldenhar complex with atypical associated anomalies: is the spectrum still widening? J. Craniofac. Surg. 17 (July (4)) (2006) 669–672. [30] A.W. James, K. Culver, B. Hall, M. Golabi, Bifid tongue: a rare feature associated with infants of diabetic mother syndrome, Am. J. Med. Genet. A 143A (2007) 2035–2039. ¨ . Deren, E.G. U ¨ tine, Prenatally diagnosed lethal type Larsen[31] D. Orhan, S. Balci, O like syndrome associated with bifid tongue, Turk. J. Pediatr. 50 (2008) 395–399. [32] N.N. Andrade, K. Raikwar, Congenital benign teratoma of the tongue with bifid tip, ankyloglossia and polydactyly: report of a case, Br. J. Oral Maxillofac. Surg. 48 (2010) e29–e30. [33] M.M. Ryan, D.B. Vuksanovic, N. Lindor, Bifid tongue, corneal clouding, and dandywalker malformation in a male infant with otopalatodigital syndrome type 2, Am. J. Med. Genet. A 155 (2011) 855–859. [34] L.P. Martin, M.M. Perez, E.G. Garcia, J.G. Martin-Moro, J.I.R. Gonzalez, M.B. Garcia, Atypical case of congenital maxillomandibular fusion with duplication of the craniofacial midline, Craniomaxillofac. Trauma Reconstr. 4 (June (2)) (2011) 113–120. [35] G.S. Sarode, R.S. Desai, S.C. Sarode, M.A. Kulkarni, Van der woude syndrome with an unusual intraoral finding, Indian J. Dent. Res. 22 (2011) 164–165. [36] R. Halli, Y. Kini, V. Kharkar, B. Rudagi, M. Hebbale, Treatment of midline cleft of the mandible-A 2-stage approach, J. Craniofac. Surg. 22 (January (1)) (2012). [37] C. Bonet, D. Penarrocba-Oltra, J.M. Minguez, B. Vera-Sirera, M. Penarrocbe-Diago, M. Penarrocha-Diago, Oral teratomas: a report of 5 cases, J. Oral Maxillofac. Surg. (2012). [38] N. Sharma, S. Passi, Goldenhar syndrome, Indian J. Dent. Res. 24 (2013) 149. [39] I. Panigrahi, R.R. Das, K.P. Kulkarni, R.K. Marwaha, Overlapping phenotypes in OFD type II and OFD type VI: report of two cases, Clin. Dysmorphol. 22 (3) (2013). [40] H.L. Wang, F.Y. Chiang, C.F. Tai, K.B. Tsai, L.F. Wang, Lingual leiomyomatous hamartoma with bifid tip and ankyloglossia in a patient without oral–facial– digital syndrome: a case report and literature review, World J. Surg. Oncol. 11 (2013) 230. [41] M.G. Gonzalez, M.P. Castro, D.V. Nieto, J.C. Bouzan, Oral–facial–digital syndrome type I: surgical approach and a case report, J. Plast. Reconstr. Aesthet. Surg. 67 (2014) 396–398.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Review Article
Accessory tongue: Classification and report of a case M. Hiradfar a,*, M. Bakhshaee a, R. Shojaeian a, N. Zabolinejad a, M. Forghani a, F. Mirhosseini b a b
Mashhad University of Medical Sciences, Iran Center of Pediatric Surgery, Iran
A R T I C L E I N F O
A B S T R A C T
Article history: Received 7 January 2015 Accepted 19 April 2015 Available online 11 May 2015
Background: Accessory tongue (AT) is a rare congenital anomaly. Due to rarity of AT, various terminologies are applied, including accessory tongue, bifid tongue, double tongue, cleft tongue, and supernumerary tongue. It seems that the anomaly has geographic distribution and most reported cases are from India and Middle East. No comprehensive classification has been introduced yet. So, we present a classification for AT according to review of all papers and documents that we found, and report a 2month-old male infant with this anomaly. ß 2015 Elsevier Ireland Ltd. All rights reserved.
Keywords: Accessory tongue Congenital anomaly Pediatric Cleft mandible
Contents 1. 2. 3. 4. 5.
Introduction . . . . . . . . . . . . . . . . . . . . . Report of the case and review article . Result . . . . . . . . . . . . . . . . . . . . . . . . . . Discussion . . . . . . . . . . . . . . . . . . . . . . Comment . . . . . . . . . . . . . . . . . . . . . . . Acknowledgements . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . .
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1. Introduction During the fourth week of intrauterine phase, the tongue is originated from the first three or four branchial arches and a median swelling (tuberculum impar) on the floor of pharynx and two lateral lingual swellings joining this central structure [1]. These three swelling are originated from the first pharyngeal arch [2]. Copula or hypobranchial eminence is another median swelling that is formed by mesoderm of the second, third, and part of the fourth branchial arch. It gives rise to posterior part of the tongue [2]. Sometimes, errors in the tongue development may occur which is due to anomalies in lingual tubercle [1,3]. Until yet, 37 cases were reported in literatures. Rarity of this anomaly, results in ill-defined terminology and classification for AT. Developmental abnormality
* Corresponding author. Tel.: +98 9151106209. E-mail address: [email protected] (M. Hiradfar). http://dx.doi.org/10.1016/j.ijporl.2015.04.029 0165-5876/ß 2015 Elsevier Ireland Ltd. All rights reserved.
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1175 1176 1176 1176 1178 1179 1179
of tongue may be major or minor, single or multiple [4]. So, many different kinds of AT could be happened with various degree of severity and functional disturbance. AT could be isolated (sporadic) or in association with other disorders (syndromic) such as, Opitz G BBB syndrome, Larsen syndrome, short rib syndrome, short rib polydactyly syndrome, oral–facial–digital, median cleft syndrome, Klipple–Feil anomaly, varadi syndrome, oro-maxillary, limb disorder, cleft palate, mandibular cleft, linea alba hernia, hypertelorism, scoliosis, aglossia, microglossia, macroglossia, hemi-glossia, Glossitis Rhombica Mediana, lingual thyroid, long tongue, and ankyloglossia. Sometimes, hamartoma or tumor (teratoma) may happen with AT simultaneously [3–10]. Sign and symptoms are variable in different age groups. Affected neonate may be completely asymptomatic at birth or suffered from breathing difficulties [11], choking [4] or swallowing disturbance [3]. Asymptomatic cases may be missed until childhood or following ages. Trouble in speech [3] may happen later in life in undiagnosed or untreated cases.
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Fig. 1. (A) Complete accessory tongue and cleft mandible. (B) OPG imaging. (C) Accessory tongue which consisted of interlacing bundles of muscular fibers and numerous serous and mucous accessory salivary glands, covered by stratified epithelium (H&E, 40). (D) Case after 5 years follow-up with good appearance and normal speech.
We report a patient with tongue duplication who was successfully treated with surgical excision and also review the previous cases to reach a novel classification. 2. Report of the case and review article A three month old female infant, a first child in a family of two, was referred to us due to failure to thrive and tongue and mandible deformity. She had a history of respiratory problem during starting feeding after birth. She hospitalized in another city due to aspiration pneumonia at one and two month old. She was delivered by cesarean section with APGAR score 8–9 and birth weight 3200 g. On admission, her weight, head circumferential, and length were 3500 g, 34 cm, and 57 cm, respectively. Her mother did not have any previous abortion, drug usage during pregnancy or diabetes induced pregnancy. In addition, no family history was found in either maternal or paternal families. In physical examination, an accessory tongue was diagnosed in left lateral side of native tongue which was slightly smaller compared to normal one (Fig. 1A). This caused the mandible to expand and deform. Also, there was evidence for double mandible bone according to OPG imaging (Fig. 1B). Lower lip had some deformity especially in mid line. At first, nasogastric tube and respiratory support were given for patient. With conservative management, breathing got better, and feeding started through nasogastric tube. Patient was discharged with good general condition after one week. Gradually, she tolerated feeding by mouth. Surgical intervention was performed in order to excise the accessory tongue with preservation of native tongue, nerves and salivary glands at age one year. Macroscopic results of accessory tongue demonstrated the resection of 4 cm 1.8 cm 1 cm accessory tongue covering by soft mucous membrane. Histopathological examination showed interlacing bundles of muscle fibers and numerous serous and mucous accessory salivary glands scattered throughout the muscle and lamina propria, covered by stratified epithelium and was consistent with normal tongue (Fig. 1C). Postoperative course was uneventful, no complication was observed and patient discharged after 4 days with good condition and ability to feed. We followed the case for 5 years and fortunately, she was growing up normally without any neurodevelopmental defect as well as with completely normal speech. Based on searching keywords (i.e., accessory tongue, cleft mandible, congenital anomaly and pediatric) through various sources like Google scholar, pubmed, and science direct, 34 articles were collected that the oldest and newest are related to 1966 and 2012. Generally, there are 38 cases that listed as following (Table 1). 3. Result According to this review, we found 37 cases with AT. 62% of cases were female. Associated anomalies were observed in 83% of
cases. The most common associated anomaly was cleft palate that was found in 43% of cases. Symptomatic and asymptomatic cases were 37% and 62%, respectively. Speech difficulty was reported in 3 cases with the prevalence of 5% and 2% in dorsal and lateral surfaces, respectively. The most prevalent associated clinical symptom was respiratory difficulties in 18% of cases. Incomplete form of AT was more dominant than complete types (55%). Complete form was mostly asymptomatic and only seen in 15%. 4. Discussion Despite of lack of genetic pattern in these cases, it seems that the prevalence of AT is more common in female gender. The exact frequency of AT is not clear in the world. Lack of proper data may be due to unavailability of good classification. Accordingly, and during our conducted surveys, we can suggest a suitable classification that helps to distinguish different aspects of this abnormality (Table 2). AT may be associated with other types of midline clefts that the most common are cleft palate and mandibular cleft. Many reported cases about congenital pathology of tongue are cystic lesions that are not related to AT. It is likely that the cysts of tongue are as a part of duplication of GIT. Given the introduction of AT as tongue duplication in some papers, it makes misunderstanding that AT can also be a subtype of GIT duplication [16–19]. Embryologically, with regard to lack of communication between construction of accessory tongue and GIT, and no reports about the simultaneity of GIT duplication in above cases, it seems that the nature of AT is completely independent and not related to GIT duplication. Because AT is placed in a group of midline defects [8], thus, monitoring the involved cases for these probable anomalies is suggested before surgical excision. The prenatal sonography is popularized now and it is possible to diagnose AT with this modality of fetus evaluation before birth [6,8]. In case of prenatal diagnosis of AT, due to probable respiratory problem just after birth, it is better to be referred mothers to more equipped departments. As polyhydramnios may be coexist with AT, prenatal sonographer must evaluate the fetus for this anomaly in these cases [9]. In postnatal period, evaluation for other midline defects is advised. We checked our case by brain MRI and OPG. No abnormality was seen in brain study despite of conducted survey by Manjila et al. [9] that shows duplication in pituitary glands. It is important to consider diabetic mothers as a predisposing factor in occurrence of AT [2,15,20]. We did not find any history of diabetes in mother of our case. In differential diagnosis, the benign lesions must be considered such as fibroma and haemangioma [1]. Until now, it is not revealed any genetic abnormalities in all the reported cases. Therefore, it seems that geographic distribution may be environmental or caused by other reasons. Rai et al. [2] described that this anomaly is more obvious in the southern coastal belt of India with the prevalence of 0.3%. Moreover, no inheritance pattern or family history has been observed.
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Table 1 Review of cases. Characteristics
Sex
Age
Sign/symptom
Localization
Complete/ incomplete
Associated anomalies
–Complete midline cleft of lower lip –Cleft palate –Dimple in the midline of the mandible –Epidermoid cyst No data
Cases Case 1
Chidzonga et al. [12]
F
Neonate
Non-symptom except for transient cyanosis
Anterior
Inc
Case 2
Comess et al. [15]
No data
No data
No data
No data
Case 3 Case 4
Widgerow [22] Chapman et al. [23]
No data F F
18-month-old –
– –
Inferolateral –
Inc –
Case 5 Case 6
Chidzonga et al. [24] Rao et al. [25]
F F
Neonate 9-h-old
– Anterior
– Inc
Case 7
Hunt and Vujanic [26]
–
Prenatal
– Aspiration pneumonia and severe respiratory distress –
–
–
Case 8
Lu et al. [7]
F
Neonate
–
Side to side
C
Case 9
Vandenhaute et al. [14]
F
–
Severe respiratory difficulties
Side to side
Inc
Cases 10 and 11
Britto et al. [11]
M
Neonate
Side to side
Inc
Case 12
Haghighi et al. [27]
F M
Neonate 1-day-old
Inferolateral Anterior
C C
Cases 13 and 14
Driva et al. [28]
F
Acute respiratory embarrassment Unable to feed adequately Respiratory difficulty and difficulty in oral feeding –
Side to side
Inc
–
Side to side
Inc
–
Inferior
Inc
–Cleft of secondary palate –Cleft palate –Cleft of the lower lip – –Midline cleft of the mandible –Micrognathia –Severe bilateral pulmonary hypoplasia –Centrally located tumor –Midline cleft of mandible and cervical vertebrae –Mild peripheral pulmonary stenosis –Linea alba hernia –Microretrognathia –Cleft palate –Intracranial tumor with extension oral cavity –U-shaped Cleft palate –Mandibular epilus –Epignathus teratoma –Cleft palate –Oral–facial–digital syndrome type 1 –Oral–facial–digital syndrome –Right mandibular and maxillary hypoplasia No associated congenital anomaly –Microretrognathia –Cleft palate –Micrognathia
Case 15
Konas et al. [29]
F
4-years3-months-old 6-years10-months-old Two-month-old
Case 16
F
18-year-old
–
Anterior
Inc
Case 17
Tewari and Shukla [3] James et al. [30]
–
10-h-old
Severe respiratory distress
Side to side
Inc
Case 18
Orhan et al. [31]
–
Prenatal
–
–
–
Cases 19 and 20
Bhattacharya et al. [4]
F
9-month-old
Choking during feeding
Anterolateral
Inc
F
19-year-old
Speech impairment
Dorsal
Inc
Dorsal
Inc
Side to side
Inc
No associated congenital defects
Dorsal
Inc
–Polydactyly –Micrognathia –Cleft palate – –Cleft lip –Bilateral cleft palate –Ankyloglossia –Van der woude syndrome –Incomplete median cleft of the lower lip –Complete median mandibular cleft –Ankyloglossia –Midline cleft of the lower lip and the mandible
F
Case 21
Kumar et al. [1]
M
28-year-old
Case 22
Surej et al. [5]
M
45-year-old
Case 23
–
6-weeks-old
Case 24
Andrade and Raikwar [32] Ryan et al. [33]
Difficulty in swallowing and speech Difficulty in speech and unusual appearance of tongue Difficulty in swallowing
M
Infant
–
Side to side
C
Case 25 Case 26
Martin et al. [34] Sarode et al. [35]
F M
1-day-old 7-year-old
– –
– –
– –
Case 27
Halli et al. [36]
F
6-month-old
–
–
Inc
Case 28
Bhattacharyya et al. [13]
M
5-month-old
–
Side to side
C
Case 29
Bonet et al. [37]
–
–
–
–
–
–Cleft palate –Lingual thyroid –Cleft palate –Lingual hamartoma –
–
M. Hiradfar et al. / International Journal of Pediatric Otorhinolaryngology 79 (2015) 1175–1179
1178 Table 1 (Continued ) Characteristics
Sex
Age
Sign/symptom
Localization
Complete/ incomplete
Associated anomalies
–Micrognathia –Cleft palate –Pierre-Robin Syndrome No associated congenital anomaly –Cleft of the lower lip involving the chin and the mandible –Inflamed median cervical cyst –Cleft palate –Scoliosis –Duplication of pituitary gland –Bilateral cleft lower lip – –Median Cleft palate –Median cleft lip – –Intraoral hamartomas –Incomplete cleft of the median upper lip cleft –Ankyloglossia –Brachydactyly –Cleft mandible –Lip deformity
Cases Case 30
Daniel et al. [6]
–
Prenatal
Breathing difficulties
Side to side
Inc
Case 31
Tewari and Shukla [3]
F
18-year-old
–
Anterior
Inc
Case 32
Anselem et al. [8]
F
Prenatal
–
Inferior
Inc
Case 33
Manjila et al. [9]
F
11-year-old
–
Side to side
C
Case 34 Case 35
Sharma and Passi [38] Panigrahi et al. [39]
F F
17-year-old 11-year-old
– –
– –
– –
Case 36 Case 37
Wang et al. [40] Gonzales et al. [41]
M F
29-year-old Neonate
– –
Anterior –
Inc Inc
Case 38
M. Hiradfar et al.
F
4.5-month-old
Respiratory and feeding difficulties
Side to side
Inc
Fig. 2. Gross anatomy and position.
In most cases, treatment includes surgical excision. However, this procedure may not relieve all of symptoms, especially in speech impairments. In addition, reduction glossoplasty is another method to treat these patients if feasible [8]. As shown in Table 1, speech disturbance was observed in three cases with the age more than 19 year old. Therefore, it is supposed that delay in surgical intervention may cause this difficulty. Early
Table 2 Suggested Classification. A: Gross anatomy I) AT similar to normal tongue, the size is the same as normal tongue (Fig. 2A) II) AT similar to normal tongue, the size is smaller than normal one (Fig. 2B) III) Any mass or tumors unlike a normal tongue that confirmed as a tongue tissue in microscopic evaluation (Fig. 2C) B: Position I) Side to side (lateral) (Fig. 2A and B) II) Over or under the native tongue (Fig. 2C and D) C: Sign/symptom I) Asymptomatic II) Symptomatic: respiratory, swallowing, and speech symptoms
intervention in our case and perfect speech at this time may also support this hypothesis. Besides the congenital form of bifid tongue, recently due to tongue piercing, acquired form of bifid tongue as a complication of this procedure may happen that is completely different in etiology symptoms and management compared to congenital form [21].
5. Comment AT is a rare anomaly with special geographic widespread. Due to lack of classification and ill-defined terminology, the exact frequency of this congenital anomaly may be obscure yet. Our classification may help better understanding of AT. According to this review, some of the cases had respiratory or feeding problems, but it seems that the severity of these difficulties were not as much as those which required emergency neonatal surgery. It is likely that early surgical intervention prevents trouble in speech at later ages. Therefore, performing an operation before starting speaking is recommended. This policy also prevents the chance of iatrogenic trauma to adjacent organs due to unnecessary operation in neonatal period.
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Acknowledgment At the end, we are really appreciative of Mrs. Ghazaleh Azim Zadeh Khosravi for her worthy assistant in collecting patient’s data. References [1] S. Kumar, S.K. Tiwary, A.K. Khanna, An accessory tongue, Singap. Med. J. 50 (5) (2009) e172. [2] R. Rai, A.R. Rai, R. Rai, K. Bhat, B.V. Muralimanju, Prevalence of bifid tongue and ankyloglossia in South Indian population with an emphasis on its embryogenesis, Int. J. Morphol. 30 (1) (2012) 182–184. [3] M. Tewari, H.S. Shukla, A rare anomaly: an accessory tongue, Indian J. Surg. 69 (February (1)) (2007). [4] V. Bhattacharya, S. Khanna, A.S. Bashir, U. Kumar, R.S. Garbyal, Cleft palate associated with hamartomatous bifid tongue. Report of two cases, J. Plast. Reconstr. Aesthet. Surg. 62 (2009) 1442–1445. [5] K.L.K. Surej, N.M. Kurien, M.P. Sivan, Isolated congenital bifid tongue, Natl. J. Maxillofac. Surg. 1 (July–December (2)) (2010) 187–189. [6] E. Daniel-Spiegel, M. Ben-Ami, Bifid tongue, a rare congenital malformation, is a prenatal clue for secondary cleft palate, J. Ultrasound Med. 31 (2012) 505–509. [7] C.-Y. Lu, R.-J. Teng, J.-W. Hou, Bifid tongue associated with midline cleft palate, mandible, cervical vertebrae and linea alba, Eur. J. Pediatr. 157 (1998) 86. [8] A. Vincent-Rohfritsch, O. Anselem, G. Grange, C. Benard, G. Viot, P. Lalau, et al., Prenatal diagnosis of a cleft of the tongue, lower lip and mandible, Ultrasound Obstet. Gynecol. 39 (2012) 110–114. [9] S. Manjila, A.E. Miller, S. Vadera, K.R. Goel, R.F. Khan, C. Crowe, et al., Duplication of the pituitary gland associated with multiple blastogenesis defects: duplication of the pituitary gland (DPG)-plus syndrome. Case report and review of literature, Surg. Neurol. Int. 3 (2012) 23. [10] E.N. Emmanouil-Nikoloussi, C. Kerameos-Foroglou, Developmental malformation of human tongue and associated syndromes (review), Bull. Group Int. Rech. Sci. Stomatol. Odontol. 35 (March–June (1–2)) (1992) 5–12. [11] J.A. Britto, R.H. Ragoowansi, B.C. Sommerlad, Double tongue, intraoral anomalies, and cleft palate – case reports and a discussion of developmental pathology, Cleft Palate-Craniofac. J. 37 (July (4)) (2000). [12] M.M. Chidzonga, K.J. Shija, Congenital median cleft of the lower lip, bifid tongue with ankyloglossia, cleft palate, and submental epidermoid cyst: report of a case, J. Oral Maxillofac. Surg. 46 (1966) 809–812. [13] C.N. Bhattacharyya, K. Kalita, M. Gogoi, K.P. Deuri, Tessier 30 facial cleft, J. Indian Assoc. Pediatr. Surg. 17 (2) (2012) 75–77. [14] B. Vandenhaute, E. Leteurtre, M. Lecomte-houcke, P. Pellerin, J.P. Nuyts, J.M. Cuisset, et al., Epignathus teratoma: report of three cases with a review of the literature, Cleft Palate-Craniofac. J. 37 (January (1)) (2000). [15] L.J. Comess, P.H. Bennett, T.A. Burch, M. Miller, Congenital anomalies and diabetes in the Prima Indians of Arizona, Diabetes 18 (1969) 471–477. [16] E.A. Ameh, A.O. Jimoh, A.H. Rafindadi, S.M. Shehu, Sublingual gastric duplication cyst causing respiratory obstruction: case report, East Afr. Med. J. 77 (July (7)) (2000) 394–395. [17] M. Blanchard, N. Kadlub, S. Boudjemaa, S. Cassier, C. Garel, G. Audry, et al., Tongue cyst in children: foregut duplication, a possible diagnosis, Rev. Stomatol. Chir. Maxillofac. 113 (December (6)) (2012) 442–447. [18] E. Vimalakar Reddy, G. Ram Mohan, G. Raghavendra Prasad, G. Sathyanarayana, Lingual gastric duplication cyst in a new born, Indian J. Otolaryngol. Head Neck Surg. 63 (October (4)) (2011) 405–406.
1179
[19] S. Hambarde, P. Bendre, D. Taide, Foregut duplication cyst presenting as lingual swelling: case report and review of literature, Natl. J. Maxillofac. Surg. 2 (January (1)) (2011) 2–5. [20] A.W. James, K. Culver, B. Hall, M. Golabi, Bifid tongue: a rare feature associated with infants of diabetic mother syndrome, Am. Med. Genet. A 143A (2007) 2035–2039. [21] P.S. Fleming, T.R. Flood, Bifid tongue – a complication of tongue piercing, Br. Dent. J. 198 (March (5)) (2005). [22] A.D. Widgerow, Klipple–Feil anomaly, cleft palate, and bifid tongue, Ann. Plast. Surg. 25 (September (3)) (1990). [23] S. Chapman, H. Goldin, 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 angenesis of the anterior arch of atlas, Br. J. Oral Mexillofac. Surg. 29 (1991) 279–281. [24] M.M. Chidzonga, V.M. Lopez Perez, S. Mzezewa, Treatment of median cleft of the lower lip, mandible and bifid tongue with ankyloglossia, Int. J. Oral Maxillofac. Surg. 25 (1996) 272–273. [25] S. Rao, S. Oak, M. Wagh, B. Kulkarni, Congenital midline palatomandibular bony fusion with a mandibular cleft and a bifid tongue, Br. J. Plast. Surg. 50 (1997) 139–141. [26] N.C.A. Hunt, G.M. Vujanic, Fibrochondrogenesis in a 17-week fetus: a case expanding the phenotype, Am. J. Med. Genet. 75 (2015) 326–329. [27] K. Haghighi, 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. [28] T. Driva, D. Franklin, P.J.M. Crawford, Variation in expression of oral–facial–digital syndrome (type I): report of two cases, Int. J. Paediatr. Dent. 14 (2004) 61–68. [29] E. Konas, H.I. Canter, M.E. Mavili, Goldenhar complex with atypical associated anomalies: is the spectrum still widening? J. Craniofac. Surg. 17 (July (4)) (2006) 669–672. [30] A.W. James, K. Culver, B. Hall, M. Golabi, Bifid tongue: a rare feature associated with infants of diabetic mother syndrome, Am. J. Med. Genet. A 143A (2007) 2035–2039. ¨ . Deren, E.G. U ¨ tine, Prenatally diagnosed lethal type Larsen[31] D. Orhan, S. Balci, O like syndrome associated with bifid tongue, Turk. J. Pediatr. 50 (2008) 395–399. [32] N.N. Andrade, K. Raikwar, Congenital benign teratoma of the tongue with bifid tip, ankyloglossia and polydactyly: report of a case, Br. J. Oral Maxillofac. Surg. 48 (2010) e29–e30. [33] M.M. Ryan, D.B. Vuksanovic, N. Lindor, Bifid tongue, corneal clouding, and dandywalker malformation in a male infant with otopalatodigital syndrome type 2, Am. J. Med. Genet. A 155 (2011) 855–859. [34] L.P. Martin, M.M. Perez, E.G. Garcia, J.G. Martin-Moro, J.I.R. Gonzalez, M.B. Garcia, Atypical case of congenital maxillomandibular fusion with duplication of the craniofacial midline, Craniomaxillofac. Trauma Reconstr. 4 (June (2)) (2011) 113–120. [35] G.S. Sarode, R.S. Desai, S.C. Sarode, M.A. Kulkarni, Van der woude syndrome with an unusual intraoral finding, Indian J. Dent. Res. 22 (2011) 164–165. [36] R. Halli, Y. Kini, V. Kharkar, B. Rudagi, M. Hebbale, Treatment of midline cleft of the mandible-A 2-stage approach, J. Craniofac. Surg. 22 (January (1)) (2012). [37] C. Bonet, D. Penarrocba-Oltra, J.M. Minguez, B. Vera-Sirera, M. Penarrocbe-Diago, M. Penarrocha-Diago, Oral teratomas: a report of 5 cases, J. Oral Maxillofac. Surg. (2012). [38] N. Sharma, S. Passi, Goldenhar syndrome, Indian J. Dent. Res. 24 (2013) 149. [39] I. Panigrahi, R.R. Das, K.P. Kulkarni, R.K. Marwaha, Overlapping phenotypes in OFD type II and OFD type VI: report of two cases, Clin. Dysmorphol. 22 (3) (2013). [40] H.L. Wang, F.Y. Chiang, C.F. Tai, K.B. Tsai, L.F. Wang, Lingual leiomyomatous hamartoma with bifid tip and ankyloglossia in a patient without oral–facial– digital syndrome: a case report and literature review, World J. Surg. Oncol. 11 (2013) 230. [41] M.G. Gonzalez, M.P. Castro, D.V. Nieto, J.C. Bouzan, Oral–facial–digital syndrome type I: surgical approach and a case report, J. Plast. Reconstr. Aesthet. Surg. 67 (2014) 396–398.
