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Acute exacerbation of macroglossia
British Journal of Plastic Surgery (2005) 58, 877–880
CASE REPORT
Acute exacerbation of macroglossia E. Vlachoua,*, M. Dalala, A. Monaghanb, H. Nishikawab a
Burns and Plastics Unit, University Hospital Birmingham, Birmingham, UK Craniofacial Unit, Birmingham Children’s Hospital, Birmingham, UK
b
Received 22 June 2004; accepted 15 December 2004
KEYWORDS Macroglossia; Venous malformation; Airway obstruction; Glossectomy; Popescu sutures
Summary We report the case of a large venous malformation of the tongue, preventing the patient from being able to contain it within the oral cavity. The extent of the malformation precluded a complete surgical excision. The immediate problems were; impending airway compromise, inability to speak, eat or drink, severe discomfort due to exposure-induced dryness and an embarrassing spectacle. This case demonstrates that even malformations considered to be incurable may be managed appropriately. Q 2005 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved.
Case report Macroglossia is defined as a resting tongue that protrudes beyond the alveolar ridge. The congenital form is an uncommon, but debilitating condition as it can affect speech, lip seal, taste sensation, swallowing and appearance. The commonest causes of true macroglossia are vascular malformations, muscular hypertrophy, storage disorders and tumours.1 Although enlargement tends to be gradual over many years we report an alarmingly rapid growth of the tongue already affected by venous malformation, which occurred over a period of 24 h. The acuteness of this presentation, the potentially life-threatening complications that ensued and their management are discussed. A 75-year-old patient, was urgently referred by her GP with acute enlargement of pre-existing * Corresponding author. E-mail address: [email protected] (E. Vlachou).
macroglossia. The background history was of an extensive cavernous haemangioma affecting the tongue, floor of mouth, oropharynx and cervical/pretracheal regions with possible retrosternal extension, demonstrated on MRI and plain radiological imaging (Fig. 1). This lesion had been present since birth gradually increasing in size to the point of the patient seeking help in 1987. Carotid angiography was unfortunately complicated by a transient ischaemic attack so the investigation and any further surgical intervention were abandoned. The patient was reviewed again by the craniofacial team 2 years previously, as the tongue’s gradual increase in size was beginning to interfere with the administration of inhaled medication. There were no functional problems in terms of speech or eating. The tongue was contained within the oral cavity, protruding only in a relaxed state (Fig. 2). A decision to treat the patient conservatively was made in view of her age, a long history of chronic obstructive airways disease
S0007-1226/$ - see front matter Q 2005 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2004.12.010
878
E. Vlachou et al.
Figure 3 Figure 1
Radiological extent of vascular malformation.
with concomitant smoking and the extent of the malformation. The complications following selective angiography 15 years previously, precluded embolisation of the feeder vessels (lingual and facial arteries) as an alternative treatment option. The acute presentation was of a 48 h history of rapid tongue enlargement following a lower respiratory tract infection. On admission to accident and emergency the tongue measured 14 cm in length, 10 cm in width and was 3 cm thick (Fig. 3). Too large to be contained in the mouth it was preventing speech as well as oral intake of solids or fluids. The patient did not display any signs of upper airway obstruction or respiratory distress. A prophylactic tracheostomy was, however, performed within 5 h from presentation to protect the airway in the event of further deterioration. The procedure proved to be technically challenging in view of the pretracheal extent of the vascular malformation
Figure 2
Pre-morbid state.
Acute enlargement of the tongue.
and an anatomically short neck, allowing the exposure of only two tracheal rings. The right lower lobe consolidation diagnosed on admission was treated with intravenous antibiotics and nutritional support was ensured via fine bore nasogastric tube feeding. It was hoped that thrombosis of the affected organ’s feeding vessels would lead to ischaemia and shrinkage. Instead the tongue continued to increase in size due to the constrictive effect of the lips (Fig. 4).
Figure 4
Further growth within 24 h.
Acute exacerbation of macroglossia
879 and articulation. Upon discharge from hospital the tongue remnant could be contained within the oral cavity and the patient demonstrated adequate lip seal. Eating was of a satisfactory level and speech improved over the first year (Fig. 6).
Discussion
Figure 5
Partial glossectomy result.
The patient underwent a partial glossectomy, sparing part of the pharyngeal portion, 3 days later (Fig. 5). A large wedge, consisting of the anterior two thirds of the protruding tongue, was excised with cutting diathermy. In view of the bulk of tissue to be excised, a midline incision supplemented the inverted ‘V’ excision technique, initially described by Koele et al.10 Haemostatic Popescu sutures controlled the bleeding from the big vascular channels.2 This intratumoural ligation technique, originally described for orofacial cavernous haemangiomas, is very useful in malformations deemed unsuitable for embolisation or resection.3 The patient spent 24 h on the high dependency unit post-operatively and required a blood transfusion of five units. There were no postoperative complications of note. The tracheostomy tube was spigotted after 8 days and removed 5 days later. The speech therapists involved in the patient’s care provided instructions and exercises to facilitate swallowing
The acuteness in the presentation of such a difficult problem is the issue that we would like to highlight through this report. The pneumonia-induced cough led to a gradual increase in tongue size until it would no longer fit in the oral cavity. The oral seal then exacerbated the organ’s engorgement by compromising its venous drainage. We could only find one previous report of traumatic entrapment leading to macroglossia, as a result of trismus in a comatose patient.4 A number of recognised treatment modalities for managing vascular anomalies of the tongue and floor of the mouth are available. Surgical ablation is suitable if the lesion proves to be resectable on imaging and the patient fit for surgery. Numerous techniques of central or peripheral reduction have been described.5 Alternatively, embolisation following angiography of high flow vascular malformations is possible, alone or as an adjunct to surgery.6,7 Intralesional photocoagulation with Nd:YAG laser has been reported to achieve up to 100% debulking with good results, in carefully selected patients. 8,9 Lastly, the conservative approach has its merits. The patient in this report was originally treated conservatively in view of the extent of the vascular malformation and a poor medical background. Danger to her airway following the acute exacerbation required urgent surgery. Most techniques described in the literature are aimed at congenital macroglossia correction in children and, therefore, at preserving maximal function. This case warranted a rapid and safe debulking procedure, which involved haemostatic sutures and allowed for enough tongue remnant to ensure functional contact with the palate. It was important to rapidly gain airway control in order to prevent a catastrophic event. Enteral feeding via the nasogastric route, keeping the exposed tongue moist and surgery, aimed at alleviating the immediate problems, resulted in a satisfactory outcome.
References
Figure 6
Twelve months post-surgery.
1. Vogel JE, Mulliken JB, Kaban LB. Macroglossia: a review of the condition and a new classification. Plast Reconstr Surg 1986;78(6):715–23.
880 2. Popescu V. Intratumoural ligation in the management of orofacial cavernous haemangiomas. J Maxillofac Surg 1985; 13:99–107. 3. Tanner NS, Pickford MA. Preliminary report: intratumoural ligation as a salvage procedure for the management of lifethreatening arteriovenous malformations. Br J Plast Surg 1993;46(8):694–702. 4. Alvi A, Theodoropoulos PA. Self-inflicted traumatic macroglossia. J Laryngol Otol 1997;111(1):75–6. 5. Davalbhakta A, Lamberty BG. Technique for uniform reduction of macroglossia. Br J Plast Surg 2000;53(4):294–7. 6. Salba S, Herberteau D, Jhaveri HS, Casasco A, Aymard A, Houdart E, et al. Therapeutic approach to arteriovenous malformations of the tongue. Eur Radiol 1998;8(2):280–5. 7. Erdmann MW, Jackson JE, Davies DM, Allison DJ.
E. Vlachou et al. Multidisciplinary approach to the management of head and neck arteriovenous malformations. Ann R Coll Surg Engl 1995;77(1):53–9. 8. Clymer MA, Fortune DS, Reinish L, Toriumi DM, Werkhaven JA, Ries WR. Interstitial Nd:YAG photocoagulation for vascular malformations and haemangiomas in childhood. Arch Otolaryngol Head Neck Surg 1998;124(4): 431–6. 9. Chang CJ, Fisher DM, Chen YR. Intralesional photocoagulation of vascular anomalies of the tongue. Br J Plast Surg 1999;52(3):178–81. 10. Koele H. Results, experience and problems of the operative treatment of anomalies with reverse overbite (mandibular protrusion). Int J Oral Surg 1965;19:427–50.
CASE REPORT
Acute exacerbation of macroglossia E. Vlachoua,*, M. Dalala, A. Monaghanb, H. Nishikawab a
Burns and Plastics Unit, University Hospital Birmingham, Birmingham, UK Craniofacial Unit, Birmingham Children’s Hospital, Birmingham, UK
b
Received 22 June 2004; accepted 15 December 2004
KEYWORDS Macroglossia; Venous malformation; Airway obstruction; Glossectomy; Popescu sutures
Summary We report the case of a large venous malformation of the tongue, preventing the patient from being able to contain it within the oral cavity. The extent of the malformation precluded a complete surgical excision. The immediate problems were; impending airway compromise, inability to speak, eat or drink, severe discomfort due to exposure-induced dryness and an embarrassing spectacle. This case demonstrates that even malformations considered to be incurable may be managed appropriately. Q 2005 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved.
Case report Macroglossia is defined as a resting tongue that protrudes beyond the alveolar ridge. The congenital form is an uncommon, but debilitating condition as it can affect speech, lip seal, taste sensation, swallowing and appearance. The commonest causes of true macroglossia are vascular malformations, muscular hypertrophy, storage disorders and tumours.1 Although enlargement tends to be gradual over many years we report an alarmingly rapid growth of the tongue already affected by venous malformation, which occurred over a period of 24 h. The acuteness of this presentation, the potentially life-threatening complications that ensued and their management are discussed. A 75-year-old patient, was urgently referred by her GP with acute enlargement of pre-existing * Corresponding author. E-mail address: [email protected] (E. Vlachou).
macroglossia. The background history was of an extensive cavernous haemangioma affecting the tongue, floor of mouth, oropharynx and cervical/pretracheal regions with possible retrosternal extension, demonstrated on MRI and plain radiological imaging (Fig. 1). This lesion had been present since birth gradually increasing in size to the point of the patient seeking help in 1987. Carotid angiography was unfortunately complicated by a transient ischaemic attack so the investigation and any further surgical intervention were abandoned. The patient was reviewed again by the craniofacial team 2 years previously, as the tongue’s gradual increase in size was beginning to interfere with the administration of inhaled medication. There were no functional problems in terms of speech or eating. The tongue was contained within the oral cavity, protruding only in a relaxed state (Fig. 2). A decision to treat the patient conservatively was made in view of her age, a long history of chronic obstructive airways disease
S0007-1226/$ - see front matter Q 2005 The British Association of Plastic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2004.12.010
878
E. Vlachou et al.
Figure 3 Figure 1
Radiological extent of vascular malformation.
with concomitant smoking and the extent of the malformation. The complications following selective angiography 15 years previously, precluded embolisation of the feeder vessels (lingual and facial arteries) as an alternative treatment option. The acute presentation was of a 48 h history of rapid tongue enlargement following a lower respiratory tract infection. On admission to accident and emergency the tongue measured 14 cm in length, 10 cm in width and was 3 cm thick (Fig. 3). Too large to be contained in the mouth it was preventing speech as well as oral intake of solids or fluids. The patient did not display any signs of upper airway obstruction or respiratory distress. A prophylactic tracheostomy was, however, performed within 5 h from presentation to protect the airway in the event of further deterioration. The procedure proved to be technically challenging in view of the pretracheal extent of the vascular malformation
Figure 2
Pre-morbid state.
Acute enlargement of the tongue.
and an anatomically short neck, allowing the exposure of only two tracheal rings. The right lower lobe consolidation diagnosed on admission was treated with intravenous antibiotics and nutritional support was ensured via fine bore nasogastric tube feeding. It was hoped that thrombosis of the affected organ’s feeding vessels would lead to ischaemia and shrinkage. Instead the tongue continued to increase in size due to the constrictive effect of the lips (Fig. 4).
Figure 4
Further growth within 24 h.
Acute exacerbation of macroglossia
879 and articulation. Upon discharge from hospital the tongue remnant could be contained within the oral cavity and the patient demonstrated adequate lip seal. Eating was of a satisfactory level and speech improved over the first year (Fig. 6).
Discussion
Figure 5
Partial glossectomy result.
The patient underwent a partial glossectomy, sparing part of the pharyngeal portion, 3 days later (Fig. 5). A large wedge, consisting of the anterior two thirds of the protruding tongue, was excised with cutting diathermy. In view of the bulk of tissue to be excised, a midline incision supplemented the inverted ‘V’ excision technique, initially described by Koele et al.10 Haemostatic Popescu sutures controlled the bleeding from the big vascular channels.2 This intratumoural ligation technique, originally described for orofacial cavernous haemangiomas, is very useful in malformations deemed unsuitable for embolisation or resection.3 The patient spent 24 h on the high dependency unit post-operatively and required a blood transfusion of five units. There were no postoperative complications of note. The tracheostomy tube was spigotted after 8 days and removed 5 days later. The speech therapists involved in the patient’s care provided instructions and exercises to facilitate swallowing
The acuteness in the presentation of such a difficult problem is the issue that we would like to highlight through this report. The pneumonia-induced cough led to a gradual increase in tongue size until it would no longer fit in the oral cavity. The oral seal then exacerbated the organ’s engorgement by compromising its venous drainage. We could only find one previous report of traumatic entrapment leading to macroglossia, as a result of trismus in a comatose patient.4 A number of recognised treatment modalities for managing vascular anomalies of the tongue and floor of the mouth are available. Surgical ablation is suitable if the lesion proves to be resectable on imaging and the patient fit for surgery. Numerous techniques of central or peripheral reduction have been described.5 Alternatively, embolisation following angiography of high flow vascular malformations is possible, alone or as an adjunct to surgery.6,7 Intralesional photocoagulation with Nd:YAG laser has been reported to achieve up to 100% debulking with good results, in carefully selected patients. 8,9 Lastly, the conservative approach has its merits. The patient in this report was originally treated conservatively in view of the extent of the vascular malformation and a poor medical background. Danger to her airway following the acute exacerbation required urgent surgery. Most techniques described in the literature are aimed at congenital macroglossia correction in children and, therefore, at preserving maximal function. This case warranted a rapid and safe debulking procedure, which involved haemostatic sutures and allowed for enough tongue remnant to ensure functional contact with the palate. It was important to rapidly gain airway control in order to prevent a catastrophic event. Enteral feeding via the nasogastric route, keeping the exposed tongue moist and surgery, aimed at alleviating the immediate problems, resulted in a satisfactory outcome.
References
Figure 6
Twelve months post-surgery.
1. Vogel JE, Mulliken JB, Kaban LB. Macroglossia: a review of the condition and a new classification. Plast Reconstr Surg 1986;78(6):715–23.
880 2. Popescu V. Intratumoural ligation in the management of orofacial cavernous haemangiomas. J Maxillofac Surg 1985; 13:99–107. 3. Tanner NS, Pickford MA. Preliminary report: intratumoural ligation as a salvage procedure for the management of lifethreatening arteriovenous malformations. Br J Plast Surg 1993;46(8):694–702. 4. Alvi A, Theodoropoulos PA. Self-inflicted traumatic macroglossia. J Laryngol Otol 1997;111(1):75–6. 5. Davalbhakta A, Lamberty BG. Technique for uniform reduction of macroglossia. Br J Plast Surg 2000;53(4):294–7. 6. Salba S, Herberteau D, Jhaveri HS, Casasco A, Aymard A, Houdart E, et al. Therapeutic approach to arteriovenous malformations of the tongue. Eur Radiol 1998;8(2):280–5. 7. Erdmann MW, Jackson JE, Davies DM, Allison DJ.
E. Vlachou et al. Multidisciplinary approach to the management of head and neck arteriovenous malformations. Ann R Coll Surg Engl 1995;77(1):53–9. 8. Clymer MA, Fortune DS, Reinish L, Toriumi DM, Werkhaven JA, Ries WR. Interstitial Nd:YAG photocoagulation for vascular malformations and haemangiomas in childhood. Arch Otolaryngol Head Neck Surg 1998;124(4): 431–6. 9. Chang CJ, Fisher DM, Chen YR. Intralesional photocoagulation of vascular anomalies of the tongue. Br J Plast Surg 1999;52(3):178–81. 10. Koele H. Results, experience and problems of the operative treatment of anomalies with reverse overbite (mandibular protrusion). Int J Oral Surg 1965;19:427–50.