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Lymphangioma of the tongue presenting as Ludwig’s angina
International Journal of Pediatric Otorhinolaryngology 51 (1999) 201 – 205 www.elsevier.com/locate/ijporl
Case report
Lymphangioma of the tongue presenting as Ludwig’s angina R.A. Tasca, H.M. Myatt *, E.J. Beckenham Department of Otorhinolaryngology, The New Children’s Hospital, Westmead, NSW 2145, Australia Received 31 March 1999; received in revised form 31 August 1999; accepted 1 September 1999
Abstract In this paper we present a case of a 6-year-old child who presented with Ludwig’s angina caused by an infected tongue base lymphangioma. We provide a literature review of tongue lymphangioma and Ludwig’s angina in the paediatric population. Both Ludwig’s angina and tongue base lymphangioma are rare conditions in their own right and a combination of the two has not previously been described in the literature. © 1999 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Lymphangioma; Ludwig’s angina; Bacterial infection; Child; Tongue disease
1. Introduction Lymphangiomas are uncommon congenital hamartomas of the lymphatic system, usually diagnosed in infancy and early childhood as lobulated masses or cystic lesions found in the head and neck, axilla and abdomen [1]. Although most commonly found in the head and neck, lymphangiomas of the tongue are rare [2,3]. Using the Landing and Farber classification, tongue lymphangiomas are usually of a cavernous type characterised by large dilated lymphatic channels which can invade surrounding tissues [2 – 4]. Ludwig’s angina is an aggressive, life threatening, rapidly spreading cellulitis of the floor of * Corresponding author. Present address: 42 Davenant Road, Oxford, OX2 8BY, UK.
mouth involving the sublingual, submental and submandibular spaces bilaterally. It presents without abscess formation and spreads along fascial planes [5,6]. Although predominantly seen in young and middle aged males, the condition has also been described in a small number of children [7–10]. In this paper we present a rare and interesting case of a child with an infected tongue lymphangioma presenting with all the features of Ludwig’s angina. 2. Case report A 6-year-old girl was transferred from a district hospital with a 3-day history of sore throat and worsening submandibular and submental swelling.
0165-5876/99/$ - see front matter © 1999 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 9 9 ) 0 0 2 6 2 - 1
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R.A. Tasca et al. / Int. J. Pediatr. Otorhinolaryngol. 51 (1999) 201–205
On presentation she was febrile (38.7°C) and found to have an erythematous, non-fluctuant swelling of the submental and submandibular triangles (Fig. 1). The floor of the mouth was raised due to gross oedema (Fig. 2) and the tongue was swollen. She had no respiratory distress and her oxygen saturation was above 98%.
She was admitted to the Intensive Care Unit for close observation because of the risk of airway obstruction. Dental examination and orthopantomogram on admission were normal. An ultrasound of the submental and submandibular triangle showed an ovoid multinodular mass lesion in the sublingual region of possible
Fig. 1. This photograph of the 6-year-old girl taken on admission shows a large submental swelling. The child is toxic and unwell.
Fig. 2. This ultrasound image of the floor of mouth shows oedema in the tissue planes and an area of lucency in the area between the points labelled as ‘2’.
R.A. Tasca et al. / Int. J. Pediatr. Otorhinolaryngol. 51 (1999) 201–205
203
Fig. 3. An intra operative photograph of the grossly oedematous epiglottis obstructing a view of the vocal cords and laryngeal inlet.
inflammatory origin raising the possibility of a pre-existing lymphatic or other form of vascular malformation. There was no abscess formation evident on the ultrasound. She was commenced on intravenous penicillin, gentamicin and metronidazole after discussion with the microbiologist. After 48 h of intravenous antibiotics there was little clinical reduction in the size of the submental swelling although the child’s general condition had improved. A decision was made to perform needle aspiration of the swelling for bacteriology and to exclude pockets of trapped pus. Intubation was difficult due to the grossly swollen epiglottis and obscured view of the glottis (Fig. 3). A Boyle Davis gag was inserted and the tongue base biopsied as it was friable and necrotic. Laryngoscopy showed a grossly oedematous epiglottis. Careful dental examination by a maxillo facial surgeon was normal. Multiple needle punctures through the neck into the floor of the mouth drew old haematoma but no free pus. Specimens were sent for both microbiology and histopathology. In view of the grossly swollen epiglottis the patient was kept intubated post operatively.
She was successfully extubated 24 h later and continued to improve on the intravenous antibiotics prescribed which were continued for a period of 5 days. Cultures of the specimens sent from theatre grew Staphylococcus aureus sensitive to flucloxacillin. Histopathological examination of the tongue base biopsies showed tissue consisting of squamous mucosa with multiple dilated endothelial lined spaces. These contained eosinophilic material, some lymphocytes, focal lymphoid aggregates and follicle formation consistent with lymphatic vascular malformations (Fig. 4). A magnetic resonance imaging scan (MRI) of the head and neck was arranged to define the extent of the lymphangioma. The study confirmed it to be localized to the tongue, with no pharyngeal or neck extension (Fig. 5). The child was discharged from hospital on oral antibiotics (Augmentin 250 mg tds) and reviewed as an outpatient 1 month later. There was no evidence of facial swelling and indirect laryngoscopy showed no abnormality of the base of the tongue, epiglottis, valleculae or larynx. No further treatment for the lymphangioma is planned as the child remains asymptomatic, the lesion had no
204
R.A. Tasca et al. / Int. J. Pediatr. Otorhinolaryngol. 51 (1999) 201–205
Fig. 4. A photograph of the histology of the tongue base biopsies stained with haematoxolin and eosin and magnified ten times. It shows squamous mucosa with dilated endothelial lined spaces. These contain eosinophilic material, lymphocytes, focal lymphoid aggregates and follicle formation consistent with lymphatic vascular malformations.
surface component and the tongue had returned to normal size. It was felt that surgical excision would involve resecting a significant portion of the tongue base and would probably not prevent further infections. She continues to be followed up in the outpatient department.
3. Discussion There are various conditions which may affect the tongue in the paediatric population namely trauma, haemangioma, lingual thyroid, amyloidosis, neurofibromatosis, carbohydrate storage disease, congenital hypothyroidism and lymphangioma. Lymphangiomas usually involve the dorsum of the tongue. They are rare lesions that need to be recognised early for an optimal outcome [1,3]. They have been classified histologically into four groups: lymphangioma simplex, cavernous lymphangioma, cystic hygroma and lymphangiosarcoma. However they can also be thought as a continuum of the same pathologic process [3]. The natural history of lymphangiomas is one of recurrent tongue enlargement secondary to bacte-
Fig. 5. A coronal plane T2 weighted MRI scan showing a large area of lucency within the substance of the tongue associated with some enhancement with Gadolinium indicating active infection.
R.A. Tasca et al. / Int. J. Pediatr. Otorhinolaryngol. 51 (1999) 201–205
rial infection, trauma, maceration and bleeding [2,8]. Management has to address the possibility of acute respiratory obstruction. Other symptoms may include drooling, poor speech, difficulty in chewing and swallowing and orthodontic abnormalities [2]. Several methods of treatment have been proposed, such as radiation therapy, electrocoagulation, cryosurgery, injection of steroids or sclerosant agents (e.g. bleomycin), surface CO2 laser photocoagulation with various degrees of success and associated complications [2,3]. The treatment of choice for neck lymphangioma is early, conservative surgical excision with preservation of vital structures. Tongue lymphangioma causing glossoptosis is best treated with laser debulking. The aim is to restore adequate breathing and swallowing, whilst leaving a sensitive tongue capable of normal speech and taste, resulting in normal orofacial development and a good cosmetic result [1,2,4]. Ludwig’s angina is a term used to define a form of cellulitis of the floor of the mouth. The inflammation usually arises bilaterally in the cellular tissue around the submandibular gland and spreads to involve both sublingual and submandibular spaces. It spreads along fascial planes and not by lymphatics, so the local lymph nodes remain unaffected. It leads to the formation of serosanguineous fluid and gangrene of the tissues, but little or no pus. The sublingual swelling is hard, non fluctuant and retains remarkable evenness and symmetry. The floor of the mouth is elevated and there can be extreme difficulty with speech, deglutition and respiration [10]. Odontogenic infections usually involving the 2nd and 3rd mandibular molars have been found to be the causes in 50–90% of cases, with oral cavity trauma and idiopathic causes accounting for the rest [5,8]. Ludwig’s angina has also been reported in immunologically compromised patients [7], and in association with glomerulonephritis, SLE and diabetes mellitus [7,8]. A wide range of micro organisms, both gram positive and negative, aerobic or anaerobic have been isolated in pure or mixed culture, with both haemolytic and non .
205
haemolytic streptococci predominating. The S. aureus reported in our case is not an unusual organism. All patients with Ludwig’s angina should be hospitalized for observation and treatment because of the risk of upper airway obstruction. An adequate airway must be maintained at all times which may require intubation (often very difficult) or tracheostomy. Many authors have advocated initial medical treatment with intravenous broad spectrum penicillin, a cephalosporin, or clindamycin [5,7,8,10]. If there is no resolution after 48 h of systemic antibiotics or suppuration becomes present, surgical incisions either intraorally and/or extraorally can be made to relieve tissue tension and provide drainage [8,10]. In summary, when a child presents with floor of mouth swelling in the absence of dental or minor salivary gland disease, a tongue lymphangioma should be considered.
References [1] M.E. Brock, R.J.H. Smith, S.E. Parey, D.L. Mobley, Lymphangioma. An otolaryngologic perspective, Int. J. Paediatr. Otorhinolaryngol. 14 (1987) 133 – 134. [2] A. Balakrishan, C.M. Bailey, Lymphangioma of the tongue. A review of pathogenesis, treatment and the use of surface laser photocoagulation, J. Laryngol. Otol. 105 (1991) 924 – 930. [3] B. Lobitz, T. Lang, Lymphangioma of the tongue, Pediatr. Emerg. Care 11 (3) (1995) 183 – 185. [4] F.T. Velcek, D.H. Klotz, L.E. Ladogana, P.K. Kottmeier, Tongue lesions in children, J. Pediatr. Surg. 14 (3) (1979) 238 – 246. [5] D.W. Megran, D.W. Scheifele, A.W. Chow, Odontogenic infections, Pediatr. Infect. Dis. 3 (3) (1984) 257 – 265. [6] B.R. Pynn, T. Sands, M.J. Pharoah, Odontogenic infections: anatomy and radiology, Oral Health 85 (5) (1995) 7 – 21. [7] R.M. Barkin, S.L. Bonis, R.M. Elghammer, J.K. Todd, Ludwig angina in children, J. Paediatr. 87 (4) (1975) 563 – 565. [8] S.J. Gross, P.I. Nieburg, Ludwig angina in childhood, Am. J. Dis. Child. 131 (1977) 291 – 292. [9] M. Kurien, M.J. Job, N. Zachariah, Ludwig’s angina, Clin. Otolaryngol. 22 (1997) 263 – 265. [10] H.H. Weisengreen, Ludwig’s angina: Historical review and reflections, Ear Nose Throat J. 65 (1986) 457 – 461.
Case report
Lymphangioma of the tongue presenting as Ludwig’s angina R.A. Tasca, H.M. Myatt *, E.J. Beckenham Department of Otorhinolaryngology, The New Children’s Hospital, Westmead, NSW 2145, Australia Received 31 March 1999; received in revised form 31 August 1999; accepted 1 September 1999
Abstract In this paper we present a case of a 6-year-old child who presented with Ludwig’s angina caused by an infected tongue base lymphangioma. We provide a literature review of tongue lymphangioma and Ludwig’s angina in the paediatric population. Both Ludwig’s angina and tongue base lymphangioma are rare conditions in their own right and a combination of the two has not previously been described in the literature. © 1999 Elsevier Science Ireland Ltd. All rights reserved. Keywords: Lymphangioma; Ludwig’s angina; Bacterial infection; Child; Tongue disease
1. Introduction Lymphangiomas are uncommon congenital hamartomas of the lymphatic system, usually diagnosed in infancy and early childhood as lobulated masses or cystic lesions found in the head and neck, axilla and abdomen [1]. Although most commonly found in the head and neck, lymphangiomas of the tongue are rare [2,3]. Using the Landing and Farber classification, tongue lymphangiomas are usually of a cavernous type characterised by large dilated lymphatic channels which can invade surrounding tissues [2 – 4]. Ludwig’s angina is an aggressive, life threatening, rapidly spreading cellulitis of the floor of * Corresponding author. Present address: 42 Davenant Road, Oxford, OX2 8BY, UK.
mouth involving the sublingual, submental and submandibular spaces bilaterally. It presents without abscess formation and spreads along fascial planes [5,6]. Although predominantly seen in young and middle aged males, the condition has also been described in a small number of children [7–10]. In this paper we present a rare and interesting case of a child with an infected tongue lymphangioma presenting with all the features of Ludwig’s angina. 2. Case report A 6-year-old girl was transferred from a district hospital with a 3-day history of sore throat and worsening submandibular and submental swelling.
0165-5876/99/$ - see front matter © 1999 Elsevier Science Ireland Ltd. All rights reserved. PII: S 0 1 6 5 - 5 8 7 6 ( 9 9 ) 0 0 2 6 2 - 1
202
R.A. Tasca et al. / Int. J. Pediatr. Otorhinolaryngol. 51 (1999) 201–205
On presentation she was febrile (38.7°C) and found to have an erythematous, non-fluctuant swelling of the submental and submandibular triangles (Fig. 1). The floor of the mouth was raised due to gross oedema (Fig. 2) and the tongue was swollen. She had no respiratory distress and her oxygen saturation was above 98%.
She was admitted to the Intensive Care Unit for close observation because of the risk of airway obstruction. Dental examination and orthopantomogram on admission were normal. An ultrasound of the submental and submandibular triangle showed an ovoid multinodular mass lesion in the sublingual region of possible
Fig. 1. This photograph of the 6-year-old girl taken on admission shows a large submental swelling. The child is toxic and unwell.
Fig. 2. This ultrasound image of the floor of mouth shows oedema in the tissue planes and an area of lucency in the area between the points labelled as ‘2’.
R.A. Tasca et al. / Int. J. Pediatr. Otorhinolaryngol. 51 (1999) 201–205
203
Fig. 3. An intra operative photograph of the grossly oedematous epiglottis obstructing a view of the vocal cords and laryngeal inlet.
inflammatory origin raising the possibility of a pre-existing lymphatic or other form of vascular malformation. There was no abscess formation evident on the ultrasound. She was commenced on intravenous penicillin, gentamicin and metronidazole after discussion with the microbiologist. After 48 h of intravenous antibiotics there was little clinical reduction in the size of the submental swelling although the child’s general condition had improved. A decision was made to perform needle aspiration of the swelling for bacteriology and to exclude pockets of trapped pus. Intubation was difficult due to the grossly swollen epiglottis and obscured view of the glottis (Fig. 3). A Boyle Davis gag was inserted and the tongue base biopsied as it was friable and necrotic. Laryngoscopy showed a grossly oedematous epiglottis. Careful dental examination by a maxillo facial surgeon was normal. Multiple needle punctures through the neck into the floor of the mouth drew old haematoma but no free pus. Specimens were sent for both microbiology and histopathology. In view of the grossly swollen epiglottis the patient was kept intubated post operatively.
She was successfully extubated 24 h later and continued to improve on the intravenous antibiotics prescribed which were continued for a period of 5 days. Cultures of the specimens sent from theatre grew Staphylococcus aureus sensitive to flucloxacillin. Histopathological examination of the tongue base biopsies showed tissue consisting of squamous mucosa with multiple dilated endothelial lined spaces. These contained eosinophilic material, some lymphocytes, focal lymphoid aggregates and follicle formation consistent with lymphatic vascular malformations (Fig. 4). A magnetic resonance imaging scan (MRI) of the head and neck was arranged to define the extent of the lymphangioma. The study confirmed it to be localized to the tongue, with no pharyngeal or neck extension (Fig. 5). The child was discharged from hospital on oral antibiotics (Augmentin 250 mg tds) and reviewed as an outpatient 1 month later. There was no evidence of facial swelling and indirect laryngoscopy showed no abnormality of the base of the tongue, epiglottis, valleculae or larynx. No further treatment for the lymphangioma is planned as the child remains asymptomatic, the lesion had no
204
R.A. Tasca et al. / Int. J. Pediatr. Otorhinolaryngol. 51 (1999) 201–205
Fig. 4. A photograph of the histology of the tongue base biopsies stained with haematoxolin and eosin and magnified ten times. It shows squamous mucosa with dilated endothelial lined spaces. These contain eosinophilic material, lymphocytes, focal lymphoid aggregates and follicle formation consistent with lymphatic vascular malformations.
surface component and the tongue had returned to normal size. It was felt that surgical excision would involve resecting a significant portion of the tongue base and would probably not prevent further infections. She continues to be followed up in the outpatient department.
3. Discussion There are various conditions which may affect the tongue in the paediatric population namely trauma, haemangioma, lingual thyroid, amyloidosis, neurofibromatosis, carbohydrate storage disease, congenital hypothyroidism and lymphangioma. Lymphangiomas usually involve the dorsum of the tongue. They are rare lesions that need to be recognised early for an optimal outcome [1,3]. They have been classified histologically into four groups: lymphangioma simplex, cavernous lymphangioma, cystic hygroma and lymphangiosarcoma. However they can also be thought as a continuum of the same pathologic process [3]. The natural history of lymphangiomas is one of recurrent tongue enlargement secondary to bacte-
Fig. 5. A coronal plane T2 weighted MRI scan showing a large area of lucency within the substance of the tongue associated with some enhancement with Gadolinium indicating active infection.
R.A. Tasca et al. / Int. J. Pediatr. Otorhinolaryngol. 51 (1999) 201–205
rial infection, trauma, maceration and bleeding [2,8]. Management has to address the possibility of acute respiratory obstruction. Other symptoms may include drooling, poor speech, difficulty in chewing and swallowing and orthodontic abnormalities [2]. Several methods of treatment have been proposed, such as radiation therapy, electrocoagulation, cryosurgery, injection of steroids or sclerosant agents (e.g. bleomycin), surface CO2 laser photocoagulation with various degrees of success and associated complications [2,3]. The treatment of choice for neck lymphangioma is early, conservative surgical excision with preservation of vital structures. Tongue lymphangioma causing glossoptosis is best treated with laser debulking. The aim is to restore adequate breathing and swallowing, whilst leaving a sensitive tongue capable of normal speech and taste, resulting in normal orofacial development and a good cosmetic result [1,2,4]. Ludwig’s angina is a term used to define a form of cellulitis of the floor of the mouth. The inflammation usually arises bilaterally in the cellular tissue around the submandibular gland and spreads to involve both sublingual and submandibular spaces. It spreads along fascial planes and not by lymphatics, so the local lymph nodes remain unaffected. It leads to the formation of serosanguineous fluid and gangrene of the tissues, but little or no pus. The sublingual swelling is hard, non fluctuant and retains remarkable evenness and symmetry. The floor of the mouth is elevated and there can be extreme difficulty with speech, deglutition and respiration [10]. Odontogenic infections usually involving the 2nd and 3rd mandibular molars have been found to be the causes in 50–90% of cases, with oral cavity trauma and idiopathic causes accounting for the rest [5,8]. Ludwig’s angina has also been reported in immunologically compromised patients [7], and in association with glomerulonephritis, SLE and diabetes mellitus [7,8]. A wide range of micro organisms, both gram positive and negative, aerobic or anaerobic have been isolated in pure or mixed culture, with both haemolytic and non .
205
haemolytic streptococci predominating. The S. aureus reported in our case is not an unusual organism. All patients with Ludwig’s angina should be hospitalized for observation and treatment because of the risk of upper airway obstruction. An adequate airway must be maintained at all times which may require intubation (often very difficult) or tracheostomy. Many authors have advocated initial medical treatment with intravenous broad spectrum penicillin, a cephalosporin, or clindamycin [5,7,8,10]. If there is no resolution after 48 h of systemic antibiotics or suppuration becomes present, surgical incisions either intraorally and/or extraorally can be made to relieve tissue tension and provide drainage [8,10]. In summary, when a child presents with floor of mouth swelling in the absence of dental or minor salivary gland disease, a tongue lymphangioma should be considered.
References [1] M.E. Brock, R.J.H. Smith, S.E. Parey, D.L. Mobley, Lymphangioma. An otolaryngologic perspective, Int. J. Paediatr. Otorhinolaryngol. 14 (1987) 133 – 134. [2] A. Balakrishan, C.M. Bailey, Lymphangioma of the tongue. A review of pathogenesis, treatment and the use of surface laser photocoagulation, J. Laryngol. Otol. 105 (1991) 924 – 930. [3] B. Lobitz, T. Lang, Lymphangioma of the tongue, Pediatr. Emerg. Care 11 (3) (1995) 183 – 185. [4] F.T. Velcek, D.H. Klotz, L.E. Ladogana, P.K. Kottmeier, Tongue lesions in children, J. Pediatr. Surg. 14 (3) (1979) 238 – 246. [5] D.W. Megran, D.W. Scheifele, A.W. Chow, Odontogenic infections, Pediatr. Infect. Dis. 3 (3) (1984) 257 – 265. [6] B.R. Pynn, T. Sands, M.J. Pharoah, Odontogenic infections: anatomy and radiology, Oral Health 85 (5) (1995) 7 – 21. [7] R.M. Barkin, S.L. Bonis, R.M. Elghammer, J.K. Todd, Ludwig angina in children, J. Paediatr. 87 (4) (1975) 563 – 565. [8] S.J. Gross, P.I. Nieburg, Ludwig angina in childhood, Am. J. Dis. Child. 131 (1977) 291 – 292. [9] M. Kurien, M.J. Job, N. Zachariah, Ludwig’s angina, Clin. Otolaryngol. 22 (1997) 263 – 265. [10] H.H. Weisengreen, Ludwig’s angina: Historical review and reflections, Ear Nose Throat J. 65 (1986) 457 – 461.