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Case report macroglossia: Review and application of tongue reduction technique Bilommi R. Irhamni * Pediatric Surgery Division, Gatot Subroto Army Hospital, Jakarta, Indonesia article info Article history: Received 19 January 2015 Received in revised form 1 March 2015 Accepted 8 March 2015 Key words: Macroglossia Tongue reduction Glossectomy abstract Congenital macroglossia is uncommon condition, Enlargement can be true as seen in vascular malfor- mations or muscular enlargement. It may cause signicant symptoms in children such as sleep apnea, respiratory distress, drooling, difculty in swallowing and dysarthria. Long-standing macroglossia leads to an anterior open bite deformity, mucosal changes, exposure to potential trauma, increased incidence of upper respiratory tract infections and failure to thrive. Tongue movements, sounds and Speech articulation may also be affected. It is important to achieve uniform global reduction of the enlarged tongue for functional as well as esthetic reasons. The multiple techniques advocated for tongue reduction reveal that an ideal procedure has yet to emerge. In our case report we describe a modied reduction technique of the tongue globally preserving the taste, sensation and mobility of the tongue suitable for cases of enlargement of the tongue as in muscular hypertrophy. It can be used for repeat reductions without jeopardizing the mobility and sensibility of the tongue. Ó 2015 The Author. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). Congenital macroglossia is uncommon. Macroglossia, or increased tongue size relative to the oral cavity. Enlargement can be true as seen in vascular malformations or muscular enlargement. Macroglossia may occurs due to muscular enlargement due to hy- perplasia of muscle bers. Histological examination of the excision specimens showed no hypertrophy of muscle bers, nor fatty inltration or signicant brosis. Nerve and muscle tissue is typi- cally normal. In children, it may cause signicant symptoms such as sleep apnea, respiratory distress, drooling, difculty in swallowing and dysarthria. Long-standing macroglossia leads to an anterior open bite deformity, mucosal changes, exposure to potential trauma, increased incidence of upper respiratory tract infections and failure to thrive. Tongue movements and sounds are often affected by the macroglossia. Speech articulation may also be affected [1e4]. The indications for tongue reduction are clear. There has been no general agreement as to optimal timing of tongue reduction in in- fants. Kopriva and Classen recommended that the optimal time for tongue reduction procedures is after 6 months of age, coinciding with a reduction in the rate of tongue growth [3,4]. There are 2 primary goals of this surgery: (1) To restore the size and shape of the tongue for function. (2) To preserve the existing functions of the tongue including articulation, deglutition, and taste. It has been suggested that the reduction procedure should result in a tongue that remains behind the lower dental arch at rest, yet can wet the lips on protrusion. 1. Case report 16 month old boy brought to Gatot Subroto Army Hospital with chief complain enlargement of his tongue. His weight was 8 kg. He had a large globular tongue 5 6 2 cm and with no associated features of palpable hepatomegaly, nor other organs enlargement. At 1 year of age, he began to have feeding problems, he was not able to close his mouth properly and was constantly drooling. He had noisy breathing at rest and respiratory distress in the supine posi- tion. Hypothyroidism, Acromegaly and Beckwith-Wiedemann syndrome was excluded. We plan to do Tongue Reduction or partial glossectomy with modied Stellate/wedge surgical technique (Figs. 1e4). * Corresponding author. E-mail addresses: [email protected], [email protected]. Contents lists available at ScienceDirect Journal of Pediatric Surgery CASE REPORTS journal homepage: www.jpscasereports.com 2213-5766/Ó 2015 The Author. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). http://dx.doi.org/10.1016/j.epsc.2015.03.009 J Ped Surg Case Reports 3 (2015) 198e200

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Page 1: Case report macroglossia: Review and application of tongue ... · Macroglossia as a presentation of the Beckwith-Wiedemann syndrome. Plast Reconstr Surg 1985;75:170e5. [4] Elliott

Contents lists available at ScienceDirect

J Ped Surg Case Reports 3 (2015) 198e200

Journal of Pediatric Surgery CASE REPORTS

journal homepage: www.jpscasereports.com

Case report macroglossia: Review and application of tonguereduction technique

Bilommi R. Irhamni*

Pediatric Surgery Division, Gatot Subroto Army Hospital, Jakarta, Indonesia

a r t i c l e i n f o

Article history:Received 19 January 2015Received in revised form1 March 2015Accepted 8 March 2015

Key words:MacroglossiaTongue reductionGlossectomy

* Corresponding author.E-mail addresses: [email protected], ruankhab

2213-5766/� 2015 The Author. Published by Elsevier Inchttp://dx.doi.org/10.1016/j.epsc.2015.03.009

a b s t r a c t

Congenital macroglossia is uncommon condition, Enlargement can be true as seen in vascular malfor-mations or muscular enlargement. It may cause significant symptoms in children such as sleep apnea,respiratory distress, drooling, difficulty in swallowing and dysarthria. Long-standing macroglossia leadsto an anterior open bite deformity, mucosal changes, exposure to potential trauma, increased incidenceof upper respiratory tract infections and failure to thrive. Tongue movements, sounds and Speecharticulation may also be affected. It is important to achieve uniform global reduction of the enlargedtongue for functional as well as esthetic reasons. The multiple techniques advocated for tongue reductionreveal that an ideal procedure has yet to emerge. In our case report we describe a modified reductiontechnique of the tongue globally preserving the taste, sensation and mobility of the tongue suitable forcases of enlargement of the tongue as in muscular hypertrophy. It can be used for repeat reductionswithout jeopardizing the mobility and sensibility of the tongue.

� 2015 The Author. Published by Elsevier Inc. This is an open access article under the CC BY-NC-NDlicense (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Congenital macroglossia is uncommon. Macroglossia, orincreased tongue size relative to the oral cavity. Enlargement can betrue as seen in vascular malformations or muscular enlargement.Macroglossia may occurs due to muscular enlargement due to hy-perplasia of muscle fibers. Histological examination of the excisionspecimens showed no hypertrophy of muscle fibers, nor fattyinfiltration or significant fibrosis. Nerve and muscle tissue is typi-cally normal. In children, it may cause significant symptoms such assleep apnea, respiratory distress, drooling, difficulty in swallowingand dysarthria. Long-standing macroglossia leads to an anterioropen bite deformity, mucosal changes, exposure to potentialtrauma, increased incidence of upper respiratory tract infectionsand failure to thrive. Tongue movements and sounds are oftenaffected by the macroglossia. Speech articulation may also beaffected [1e4].

The indications for tongue reduction are clear. There has been nogeneral agreement as to optimal timing of tongue reduction in in-fants. Kopriva and Classen recommended that the optimal time fortongue reduction procedures is after 6 months of age, coincidingwith a reduction in the rate of tongue growth [3,4].

[email protected].

. This is an open access article under

� There are 2 primary goals of this surgery:

(1) To restore the size and shape of the tongue for function.

(2) To preserve the existing functions of the tongue includingarticulation, deglutition, and taste.

It has been suggested that the reduction procedure should resultin a tongue that remains behind the lower dental arch at rest, yetcan wet the lips on protrusion.

1. Case report

16 month old boy brought to Gatot Subroto Army Hospital withchief complain enlargement of his tongue. His weight was 8 kg. Hehad a large globular tongue 5 � 6 � 2 cm and with no associatedfeatures of palpable hepatomegaly, nor other organs enlargement.At 1 year of age, he began to have feeding problems, hewas not ableto close his mouth properly and was constantly drooling. He hadnoisy breathing at rest and respiratory distress in the supine posi-tion. Hypothyroidism, Acromegaly and Beckwith-Wiedemannsyndrome was excluded.

We plan to do Tongue Reduction or partial glossectomy withmodified Stellate/wedge surgical technique (Figs. 1e4).

the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Page 2: Case report macroglossia: Review and application of tongue ... · Macroglossia as a presentation of the Beckwith-Wiedemann syndrome. Plast Reconstr Surg 1985;75:170e5. [4] Elliott

Fig. 1. Partial glossectomy with Heggie technique.

Fig. 2. The clinical pictures of our patient before operation.

Fig. 3. The clinical pictures of our patient before operation.

B.R. Irhamni / J Ped Surg Case Reports 3 (2015) 198e200 199

2. Discussion

The aim in this case was to reduce the tongue globally whilepreserving the taste, sensation and mobility of the tongue. Themultiple techniques advocated for tongue reduction reveal that anideal procedure has yet to emerge [1,2,4,5].

This is understandable as the condition is relatively rare with avariation in the degree of macroglossia. While reducing the tongue,it is important to preserve the lingual nerve and hypoglossal ar-teries. Bracka’s has shown that the lingual arteries run as a pair on

the ventral surface of the tongue on either side of the mid-lineseptum. The sensory nerve for the anterior two-thirds of the tongueis the lingual nerves. It runs in the floor of the mouth together withthe artery progressing towards the tip of the tongue [1,5e8].

3. Conclusion

In our technique, provided that the excision does not encroachonto the base mid-line of the tongue to preserve the lingual nerveand hypoglossal arteries. It can be used for repeat reductions and in

Page 3: Case report macroglossia: Review and application of tongue ... · Macroglossia as a presentation of the Beckwith-Wiedemann syndrome. Plast Reconstr Surg 1985;75:170e5. [4] Elliott

Fig. 4. Operation steps of the modified heggie technique.

B.R. Irhamni / J Ped Surg Case Reports 3 (2015) 198e200200

muscular hyperplasia without jeopardizing the mobility and sen-sibility of the tongue.

References

[1] Davalbhakta A, Lamberty BGH. Technique for uniform reduction of macro-glossia. Br J Plast Surg 2000;53:294e7.

[2] Rizer Franklin M, Schechter Gary L, Richardson Mark A. Macroglossia: etiologicconsiderations and management techniques. Int J Pediatr Otorhinolaryngol1985;8:225e36.

[3] McManamny DS, Barnett JS. Macroglossia as a presentation of the Beckwith-Wiedemann syndrome. Plast Reconstr Surg 1985;75:170e5.

[4] Elliott M, Bayly R, Cole T, Temple IK, Maher ER. Clinical features and naturalhistory of Beckwith-Wiedemann syndrome: presentation of 74 new cases. ClinGenet 1994;46:168e74.

[5] Weksberg R, Shuman C, Smith AC. Beckwith-Wiedemann syndrome. Am J MedGenet C Semin Med Genet 2005;137C:12.

[6] Tomlinson JK, Morse SA, Bernard SPL, Greensmith AL, Meara JG. Long-termoutcomes of surgical tongue reduction in Beckwith-Wiedemann syndrome.Plast Reconstr Surg 2007;119:992e1002.

[7] Vogel JE, Mulliken JB, Kaban LB. Macroglossia: a review of the condition and anew classification. Plast Reconstr Surg 1986;78:715e23.

[8] Kimura Y, Kamada Y, Kimura S. Anesthetic management of two cases ofBeckwith-Wiedemann syndrome. J Anesth 2008;22:93e5.