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CASE REPORT Table of Contents   
Year : 2011  |  Volume : 8  |  Issue : 2  |  Page : 229-231
Giant macroglossia with persistent nonocclusion in a neonate


1 Department of Surgery, University of Port Harcourt Teaching Hospital, Port Harcourt, Rivers State, Nigeria
2 Department of Oral and Maxillofacial Surgery, University of Port Harcourt Teaching Hospital, Port Harcourt, Rivers State, Nigeria

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Date of Web Publication14-Oct-2011
 

   Abstract 

Excessive enlargement of the tongue, macroglossia, is a well-recognised clinical condition which can pose significant social and health problems. We report a case of a neonate who was born with a tongue grossly enlarged to the point of inability to feed and deformation of the jaw. Despite surgical reduction of the tongue to normal size, there was a persistent nonocclusion of the mouth, making sucking impossible. Physiotherapy did not help and our experience with neonatal jaw osteotomy is limited. We wonder if a prenatal aspiration of the tongue cyst could have forestalled this jaw deformation.

Keywords: Jaw deformation, macroglossia, neonate

How to cite this article:
Okoro PE, Akadiri OA. Giant macroglossia with persistent nonocclusion in a neonate. Afr J Paediatr Surg 2011;8:229-31

How to cite this URL:
Okoro PE, Akadiri OA. Giant macroglossia with persistent nonocclusion in a neonate. Afr J Paediatr Surg [serial online] 2011 [cited 2021 Dec 4];8:229-31. Available from: https://www.afrjpaedsurg.org/text.asp?2011/8/2/229/86070

   Introduction Top


Macroglossia is a clinical condition in which the tongue is not completely accommodated by the buccal cavity. It is commonly due to an excessive enlargement of the tongue (true macroglossia), but occasionally due to a relatively small buccal cavity (apparent macroglossia). True macroglossia may be due to primary condition of the tongue or a systemic disorder. A wide variety of primary conditions of the tongue can cause tongue enlargement, e.g., lingual thyroid, cysts, tumours, haemangioma, myositis, etc. [1],[2] True macroglossia arising from systemic disorders can constitute important diagnostic feature of such disorders (e.g., hypothyroidism, amyloidosis, Beckwith Wiedemann syndrome, etc.) [3],[4],[5] Apparent macroglossia may result from neurological impairment of the tongue [6] or a relatively small buccal cavity as may occur in Pierre Robin syndrome and Down's syndrome. [7] In macroglossia, the tongue protrudes between the incisors at rest. We encountered a case of extreme tongue enlargement causing a jaw deformation (nonocclusion) which persisted even after surgical reduction of tongue enlargement. We report this unusual picture and review the literature on management of such cases.


   Case Report Top


A male neonate was brought to our paediatric surgical outpatient clinic on the second day of life with marked tongue enlargement and inability to feed since birth. He had been on IV infusion of 10% dextrose water in the past 24 hours, since oral feeding was not possible due to the tongue mass. He was delivered by spontaneous vaginal delivery in the obstetrics department of same institution and the patient cried immediately after birth. A prenatal ultrasound did not pick any foetal abnormality. On examination, the patient was a male neonate in no obvious respiratory distress. He was not pale, afebrile, anicteric, acyanotic, and weighed 3.6 kg. There was a globular mass occupying the whole buccal cavity and protruding out of the mouth with stretching of the lips. Mass measured about 9 × 9 cm and did not transilluminate [Figure 1]. A gentle downward pressure on the mass confirmed that it was a tongue mass involving mostly the anterior two-thirds of the tongue. The chest was clinically clear and other systems were within normal limits. There were no features suggestive of the presence of Beckwith Wiedemann syndrome. X-ray of the temporomandibular joint was not done as we considered that it may not be informative as the involved bones will not have been sufficiently ossified at that age. The patient was operated on the third day of life under general anaesthesia with nasotracheal intubation. At surgery, a longitudinal incision along the lateral margin of the tongue was used to access the tongue mass. A unilocular cyst was identified and excised from the tongue. Stretched and redundant tongue tissues were also excised before the tongue incision was closed. The cyst had a thick wall and contained milk coloured fluid. Histology of cyst wall showed duplication cyst of the tongue with multiple lymphoid nodules in the wall. The postoperative recovery was unremarkable and there were no complications. However, the mouth could not be closed despite reduction of tongue size to near normal [Figure 2]. Maxillofacial and ENT reviews recommended physiotherapy which was commenced. The nonocclusion of the mouth, however, persisted 6 months after commencement of physiotherapy. This made sucking of breast impossible, and feeding of the patient after surgery was only possible with cup and spoon. The gaping mouth also induced glossitis due to tendency to tongue desiccation.
Figure 1: Shows mouth agape despite reduction of tongue size to normal

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Figure 2: Shows enlarged tongue with the lips stretched around it

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   Discussion Top


Macroglossia is not among the commoner oral anomalies seen in children in our environment. [8] Mild forms of it may pass unnoticed or do not require any treatment. When they require surgical treatment, it can constitute significant challenge due to limited experience, particularly with extremely enlarged tongue. Surgical correction of macroglossia is usually required when it constitutes a significant cosmetic concern, causes feeding difficulties, speech problems, airway obstruction, excessive tongue desiccation, ulceration or risk of tongue trauma. [9] Macroglossia caused by systemic conditions requires surgery occasionally, but often responds to treatment of such systemic conditions, e.g., hypothyroidism. [3] However, those due to local lesions of the tongue often require a surgical reduction of the tongue, e.g., tumours. The optimal age for surgical correction of macroglossia has been put at 4-7 years. [10] However, in our index patient, the huge size of the tongue, risk of desiccation and inability to feed made early surgical intervention imperative.

Our surgical intervention achieved reduction of tongue size, but the mouth remained agape. Such malocclusion or nonocclusion is a well-recognised feature of macroglossia. [9],[11] They commonly resolve with surgical reduction of tongue size. [12] However, in rare situations, the occlusion problem is not due to the mere presence of a large tongue but a consequent deformation of the jaw and or the temporomandibular joint. This is the situation in our case and posed an extra challenge as breast sucking was not possible and tongue desiccation was still a risk despite reduction of tongue size to normal. Physiotherapy and gentle manipulation of the jaw did not improve the nonocclusion. We had considered a jaw osteototomy but we did not have adequate experience with this procedure and its outcome in neonates. The inability of a prenatal ultrasound to detect this lesion points to the inadequacy of this investigative tool in our environment. We wonder, however, if this condition had been diagnosed prenatally, whether this deformation could have been safely forestalled by an ultrasound guided aspiration of the tongue cyst or other such prenatal intervention.


   Conclusion Top


Macroglossia with significant jaw deformation and nonocclusion has been reported. Our experience in the management of such cases involving a neonate is still limited. There are also limited reports and series on this scenario. We think that considerations may need to be made for intrauterine intervention in prenatally diagnosed cases and jaw osteotomy as part of corrective surgery. There may be need for a study to establish the best ways to manage such cases.

 
   References Top

1.Petraud A, Khonsari RH, Corre P, Mercier JM. Bronchogenic cyst of the tongue in an adult. Rev Stomatol Chir Maxillofac 2010;111:46-8.  Back to cited text no. 1
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2.Lee MW, Huh JR, Lee WJ, Choi JH, Moon KC, Koh JK. Focal myositis of the tongue presenting as macroglossia. Clin Exp Dermatol 2009;34:e869-72.  Back to cited text no. 2
[PUBMED]  [FULLTEXT]  
3.Adesanmi TA, Adimora GN, Tagbo BN. Congenital hypothyroidism: A delayed diagnosis in a neonate: A case report. Niger J Clin Pract 2009;12:208-11.  Back to cited text no. 3
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4.Molinos CS, Pesqueira FP, Diaz Peromingo JA, Lirola DA. Shoulder pad and macroglossia: Two signs of amyloidosis. Rev Clin Esp 2009;209:521-2.  Back to cited text no. 4
    
5.Spivey PS, Bradshaw WT. Recognition and management of the infant with Beckwith Wiedemann syndrome. Adv Neonatal Care 2009;9:279-84.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Holle D, Kastrup O, Sheu SY, Obermann M. Neurological picture: Tongue pseudohypertrophy in idiopathic hypoglossal nerve palsy. J Neurol Neurosurg Psychiatry 2009;80:1393.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  
7.Rizer FM, Schechter GL, Richrdson MA. Macroglossia: Etiologic considerations and management techniques. Int J Pediatr Otorhinolaryngol 1985;8:225-36.  Back to cited text no. 7
    
8.Sawyer DR, Taiwo EO, Mosadoni A. Oral anomalies in Nigerian children. Community Dent Oral Epidemiol 1984;12:264-73.  Back to cited text no. 8
    
9.Wolford LM, Cottrell DA. Diagnosis of macroglossia and indications for reduction glossectomy. Am J Orthod Dentofacial Orthop 1996;110:170-7.  Back to cited text no. 9
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10.Cotton RT, Willging JP. Airway malformations and reconstruction. In: Ashcraft KW, editor. Pediatric Surgery. 3 rd ed. Pennsylvania: WB Saunders; 2000. p. 256-72.  Back to cited text no. 10
    
11.Perkins JA. Overview of macroglossia and its treatment. Curr Opin Otolaryngol Head Neck Surg 2009;17:460-5.  Back to cited text no. 11
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12.Lacour M, Grappin G, Diop L, Astabie F. Considerable incisor gaping treated by partial glossectomy. Bull Soc Med Afr Noire Lang Fr 1970;15:332-6.  Back to cited text no. 12
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Correspondence Address:
Philemon E Okoro
Department of Surgery, Paediatric Surgery Unit, University of Port Harcourt Teaching Hospital, Port Harcourt, Rivers State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0189-6725.86070

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    Abstract
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   Case Report
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