Case Report Isolated microglossia: A case report

2 downloads 0 Views 618KB Size Report
This syndrome shows no predilection for sex and has no ... the timing of treatment but orthodontic treatment should start as soon as ... (Amniotic band syndrome).
[Downloaded free from on Tuesday, August 02, 2016, IP:]

Case Report Isolated microglossia: A case report Sanjib Singh Nepram, Pradeep Jain1, Rajshree Devi Huidrom2 Department of Plastic Surgery, Regional Institute of Medical Sciences (RIMS), Imphal, Manipur, 1Department of Plastic Surgery, Institute of Medical Sciences (IMS), Banaras Hindu University (BHU), Varanasi, Uttar Pradesh, 2Department of Anatomy, Jawaharlal Institute of Medical Sciences, Porompat, Imphal, India

Abstract A case of microglossia is reported herewith. 7 month old child had a small tongue but there were no breathing, feeding difficulties. He had a small mandible with no arch collapse. He had no dentition on the mandible but had 4 teeth on the upper jaw. Key Words: Microglossia, tongue anomalies and mandibular anomalies

CASE REPORT A 7-month-old male child was brought to the outpatient department by his mother with complaints of having a small tongue [Figure 1]. The child apparently had no problem in sucking milk and in taking semisolid food. There was no positive family history of a similar problem and the mother had no history of drug intake during her pregnancy. Further examination revealed that the child had a small-sized mandible but with no obvious arch collapse. The child had drooling of saliva. Four maxillary incisors had already erupted but surprisingly no dentition was seen on the mandible. There were no associated limb and cardiac anomalies.

DISCUSSION Microglossia is a very rare malformation. Since the first case reported by Antoine De Jussieu in 1718, there have been less than 50 cases reported so far.[1,2] This anomaly is almost always associated with malformations in the extremities, especially the hands and feet, cleft palate, and dental agenesis [Figure 2]. Isolated microglossia is even rarer. What is commonly observed in case of these malformations is a rudimentary, small tongue that stretches and lengthens if we grab it with forceps. Access this article online Quick Response Code:


Figure 1: Child with microglossia

This syndrome shows no predilection for sex and has no genetic implications. No teratogen has been detected in patients with this syndrome.[3] Its etiology must be searched for in some sort of fetal cell traumatism in the first few weeks of gestation. At the fourth week of life, the anterior two-third of the tongue develops from the mandibular arch, from two lateral swellings and a median This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. For reprints contact: [email protected]

DOI: 10.4103/0972-4958.170804

How to cite this article: Nepram SS, Jain P, Huidrom RD. Isolated microglossia: A case report. J Med Soc 2015;29:180-1.

Address for correspondence: Dr. Huidrom Rajshree Devi, Assistant Professor of Anatomy, Jawaharlal Institute of Medical Sciences, Porompat, Imphal, Manipur, India. E-mail: [email protected]


© 2015 Journal of Medical Society | Published by Wolters Kluwer - Medknow

[Downloaded free from on Tuesday, August 02, 2016, IP:]

Nepram, et al.: Isolated microglossia

Oromandibular limb hypogenesis syndrome (proposed by Chicarilli) Type I

Type II

Type III

Type IV

Micrognathia (mandibular) Pierre Robin syndrome Hanhart’s syndrome Microglossia Hypoglossia Hypoglossia hypodactyly Dysgnathia (maxillomandibular) Glossopalatine ankylosis Glossopalatine ankylosis hypodactyly Other Mobius syndrome Charlie M syndrome (Amniotic band syndrome)

Figure 2: Oromandibular limb hypogenesis syndrome

swelling, that is, the tuberculum impar. Some feel that the tuberculum impar does not make a significant contribution in the formation of the tongue. The posterior third of the tongue is formed by the mesoderm of the third and fourth branchial arches. There are various anomalies of the tongue that are as follows: 1. Microglossia, 2. Macroglossia, 3. Glossopalatine ankylosis, 4. Accessory tongue, 5. Long tongue, 6. Cleft or bifid tongue (failure of the two lateral swellings to fuse), 7. Glossitis rhombica mediana, and 8. Lingual thyroid.[4] The cause of microglossia is poorly understood. Stress is necessary for bony growth; lack of stimulus from tongue musculature may be responsible for the mandibular hypoplasia. As a consequence of the lack of muscular stimulus between the alveolar arches, these do not develop transversely and the mandible does not grow in the anterior direction, as a result producing a severe dentoskeletal malocclusion.

patient was brought to the hospital for the first time at 7 months of age and he could suck mother’s milk well. There is a report of a case of aglossia who reported for the first time at 30 years of age.[10] Hypodontia or missing mandibular incisor is a finding by other authors too.[11] Clinical understanding of the changes in the mechanisms of oral suction, mastication, swallowing, and speech, as well as the existing dental occlusion, requires a multidisciplinary team approach so that a more effective treatment plan can be designed.[12] In cases of isolated microglossia, such as ours, distraction osteogenesis and orthodontic intervention are the mainstay of treatment.[13] There is still no consensus on the timing of treatment but orthodontic treatment should start as soon as an orthodontic appliance is retained and distraction osteogenesis performed after 2 years of age.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.



5. 6.

Hall first classified oromandibular limb hypogenesis in 1971. [3] Chicarilli (1985) et al. feel that the classification of Hall lacks practicality and have suggested a classification system depending on the primary cause of the anomaly.[5] Our case fits in type 2 of this classification. The prognosis seems to be graver as the number of malformations increases. Infants with faciocardiomelic syndrome do not live for more than 3 weeks. Three cases of hypoglossia associated with situs inversus have also been reported so far.[3,6,7] Hypoglossia can also occur with maxillomandibular fusion.[8,9]


Generally, patients with these malformations do not have speech or swallowing-related problems; however, out of the cases reported by Thorp et al.[2] five required tube feeding and two required tracheostomy. In our case, the


Journal of Medical Society / Sep-Dec 2015 / Vol 29 | Issue 3

8. 9.

10. 11.


Weingarten RT, Walner DL, Holinger LD. Tongue hypoplasia in a newborn. Int J Pediatr Otorhinolaryngol 1993;25:235-41. Thorp MA, de Waal PJ, Prescott CA. Extreme microglossia. Int J Pediatr Otorhinolaryngol 2003;67:473-7. Jang GY, Lee KC, Choung JT, Son CS, Tockgo YC. Congenital aglossia with situs inversus totalis — A case report. J Korean Med Sci 1997;12:55-7. Emmanouil-Nikoloussi EN, Kerameos-Foroglou C. Developmental malformations of human tongue and associated syndromes (review). Bull Group Int Rech Sci Stomatol Odontol 1992;35:5-12. Chicarilli ZN, Polayes IM. Oromandibular limb hypogenesis syndromes. Plast Reconstr Surg 1985;76:13-24. Amor DJ, Craig JE. Situs inversus totalis and congenital hypoglossia. Clin Dysmorphol 2001;10:47-50. Oulis CJ, Thornton JB. Severe congenital hypoglossia and micrognathia with other multiple birth defects. J Oral Pathol 1982;11:276-82. Arshad AR, Goh CS. Hypoglossia congenita with anterior maxillo-mandibular fusion. Br J Plast Surg 1994;47:139-41. Zanini SA, Seara S, Willhelm R, Heitz C, Tershakowec M, da Costa AR. Hypoglossia-hypodactyly syndrome in a Brazilian child: Clinical and surgical aspects. J Craniofac Surg 1992;3:33-4. Khalil KC, Dayal PK, Gopakumar R, Prashanth S. Aglossia: A case report. Quintessence Int 1995;26:359-60. Yasuda Y, Kitai N, Fujii Y, Murakami S, Takada K. Report of a patient with hypoglossia-hypodactylia syndrome and a review of the literature. Cleft Palate Craniofac J 2003;40:196-202. Weckx LL, Justino DA, Guedes ZC, Weckx LY. Hypoglossia congenita. Ear Nose Throat J 1990;69:108, 111-3. Yamada A, Konno N, Imai Y, Saitou C, Kochi S. Treatment of hypoglossia-hypodactyly syndrome without extremity anomalies. Plast Reconstr Surg 2000;106:274-9. 181