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Congenital Frey's syndrome associated with nontraumatic bilateral trifid mandibular condyle
Int. J. Oral Maxillofac. Surg. 2013; 42: 237–239 http://dx.doi.org/10.1016/j.ijom.2012.06.016, available online at http://www.sciencedirect.com
Case Report TMJ Disorders
Congenital Frey’s syndrome associated with nontraumatic bilateral trifid mandibular condyle
J. Motta-Junior, T. G. Aita, C. L. Pereira-Stabile, G. A. V. Stabile Department of Oral and Maxillofacial Surgery, State University of Londrina, Londrina, Parana´, Brazil
J. Motta-Junior, T. G. Aita, C. L. Pereira-Stabile, G. A. V. Stabile: Congenital Frey’s syndrome associated with nontraumatic bilateral trifid mandibular condyle. Int. J. Oral Maxillofac. Surg. 2013; 42: 237–239. # 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. Congenital Frey’s syndrome and bilateral trifid mandibular condyle are two different entities. The occurrence of both together is rare and has not been reported in the literature. This article describes the case of a 17-year-old male patient who complained of bilateral warmness, flushing and sweating in the preauricular area after eating spicy and sour foods since childhood. He had no complaint related to the functions of the temporomandibular joint and had no history of facial trauma. The patient was treated with injections of botulinum toxin A and the signs and symptoms of Frey’s syndrome improved significantly.
Accepted for publication 22 June 2012 Available online 24 July 2012
Warmness, flushing and sweating in the preauricular area are signs and symptoms of Frey’s syndrome. This syndrome was first described in 1757, but its occurrence after auriculotemporal nerve damage was described in 1923 by the Polish neurologist, Łucja Frey.1 The syndrome can be associated with direct or indirect damage of the greater auricular nerve and/or the auriculotemporal nerve. An anatomically abnormal mandibular condyle is also unusual and can be associated with several situations, especially with previous trauma to the temporomandibular joint (TMJ). One of the abnormalities of the mandibular condyle is related to the number of condylar heads. The first report about this was published in 1941 by Hrdlicka.2 Only one specific case of trifid mandibular condyle associated with
There was no report of facial trauma. To support the diagnosis, orthopantomography was performed and trifid condyle heads were observed on both sides (Fig. 1). Considering the radiographic findings, a computed tomography (CT) of the mandible was performed. Three dimensional (3D) reconstruction of this examination revealed the trifid anatomy of both condyle heads and their relationship with the joint cavities (Fig. 2). Following the diagnostic imaging, Minor’s test was performed. A solution containing iodine and ethanol was applied to the preauricular area on both sides. When it was dry, corn starch powder was applied to the same areas. The patient was asked to eat a sour candy and after 30 s the preauricular areas tested positive with a bluish-black colour change because of the
0901-5027/020237 + 03 $36.00/0
a history of previous facial trauma has been reported3 and the congenital occurrence of a trifid condyle is uncommon. The aim of this paper is to report a case of congenital Frey’s syndrome associated with bilateral trifid mandibular condyle and to describe the treatment for this patient. Case report
A 17-year-old man was referred to the authors by his general dentist. His medical history was noncontributory. His complaints were bilateral warmness, flushing and sweating of the preauricular area after eating spicy and sour foods since childhood. His daily routine was particularly disturbed by this, mainly when eating in public. There was no history of the use of medication by his mother during pregnancy and his birth was reported as uneventful.
# 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
238
Motta-Junior et al.
Fig. 1. Orthopantomography displays the trifid condyle heads on both sides (arrows).
iodine–starch reaction, sweat secretion also increased (Fig. 3). For an exact determination of the hyperaesthetic regions, flushing and sweating areas were mapped and divided into 9 fields of 2 cm2 on each side. Based on the patient’s history and clinical, radiographic, 3D CT reconstruction and Minor’s test, the diagnosis of Frey’s syndrome associated with nontraumatic bilateral trifid mandibular condyle was suggested. The patient was treated conservatively with subcutaneous injection of botulinum toxin A (BTA) 25 U/0.1 ml in the centre of each field for treatment of the Frey’s syndrome. After 3 days, Minor’s test was performed again to confirm the effect of BTA; some remaining areas of sweating were observed. The patient had
no complaints after the application of BTA and has been followed-up for 2 years. Discussion
Trifid or bifid mandibular condyles are uncommon anomalies. There are two theories about this deformity. Walker4 sectioned and dislocated the condyle of monkeys and was able to show development of a new condylar head, providing evidence that fracture can cause new condyle formation. Blackwood5 advocated the theory of embryological malformation. According to this theory, a septum vascular fibre in the condylar cartilage is developed when the foetus is about 20 weeks old. After birth, in the ninth week
of life, the septum disappears and new bone formation on the condyle begins. If this septum remains, abnormal ossification can occur and cause bifid/trifid condyle development. In the present case report, the origin of the trifid condyles is probably related to embryological changes, because both condyles were similarly affected and according to the parents there was no history of facial trauma or surgery. Frey’s syndrome usually occurs in patients who have undergone submandibular or parotid salivary gland surgery6 because of the manipulation of the auriculotemporal nerve and the great auricular nerve. Another cause of this syndrome can be mandibular condyle fracture, due to the intimate anatomical relationship of the auriculotemporal nerve with the capsule of the TMJ. The most accepted theory to explain Frey’s syndrome is the theory of aberrant regeneration.7 The misdirected regeneration of parasympathetic fibres of the auriculotemporal nerve after facial trauma promotes simultaneous activation of parotid and sweat glands by acetylcholine. Another possible source for this aberrant innervation is direct contact of some great auricular nerve fibres with the auriculotemporal nerve.8 Regardless of the causes of the condyle abnormality or Frey’s syndrome, the objective of treatment must be focused on the signs and symptoms, which include
Fig. 2. 3D reconstruction showing the trifid mandible condyle: right (A) and left (B) with medial, lateral and posterior condylar heads.
Fig. 3. Positive Minor’s test. Sweating on left (A) and right (B) preauricular areas.
Congenital Frey’s syndrome and bilateral trifid mandibular condyle sweating, flushing and warmness of the cheek, retroauricular and temporal regions after eating.9 Options for treatment are surgery with skin excision and interposition of fascia lata or muscle flaps8; systemic or topical drugs with anticholinergic or antihidrotic effects; and intradermal injection of BTA.10 BTA has been shown to be effective in treating Frey’s syndrome treatment and after some applications the sweat glands of the skin can undergo atrophy. BTA prevents the release of acetylcholine in the synaptic gap of the nerves to the salivary and sweat glands. Although its action is not prolonged and its effect is temporary, it was the option of choice for treatment, because of its proven benefits.10 Besides being less invasive than surgical treatment, the costs and infection risks are diminished in BTA treatment. The patient’s age and previous treatment must also be considered when choosing between BTA and surgical treatment. This case has demonstrated that bilateral Frey’s syndrome and trifid condyle can occur simultaneously and have similar embryologic origin. Surgeons should be aware that these alterations can occur spontaneously and not only after surgery or trauma. In such cases the treatment given must consider the signs and symp-
toms and focus on promoting the patient’s wellbeing. Funding
None. Competing interests
None declared. Ethical approval
Not required. References 1. Jacobsen N, Hopkins C. The bullet that hit a nerve: the history of Lucja Frey and her syndrome. J Laryngol Otol 2006;120: 178–80. 2. Hrdlicka A. Lower jaw: double condyles. Am J Phys Anthropol 1941;28:75–89. 3. Artvinli LB, Kansu O. Trifid mandibular condyle: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;95:251–4. 4. Walker RV. Traumatic mandibular condylar fracture dislocations. Effect on growth in the Macaca rhesus monkey. Am J Surg 1960; 100:850–63.
239
5. Blackwood HJ. The double-headed mandibular condyle. Am J Phys Anthropol 1957;15:1–8. 6. Clayman MA, Clayman SM, Seagle MB. A review of the surgical and medical treatment of Frey syndrome. Ann Plast Surg 2006;57:581–4. 7. Gardner WJ, McCubbin JW. Auriculotemporal syndrome; gustatory sweating due to misdirection of regenerated nerve fibers. J Am Med Assoc 1956;160:272–7. 8. Laage-Hellman JE. Treatment of gustatory sweating and flushing. Acta Otolaryngol 1958;49:132–43. 9. Laskawi R, Ellies M, Rodel R, Schoenebeck C. Gustatory sweating: clinical implications and etiologic aspects. J Oral Maxillofac Surg 1999;57:642–8. [discussion 648–649]. 10. von Lindern JJ, Niederhagen B, Berge S, Reich RH. Treatment of Frey’s syndrome with type A botulinum toxin: case report. J Oral Maxillofac Surg 2000;58:1411–4.
Address: Joel Motta Junior Clı´nica Odontolo´gica Universita´ria Rua Pernambuco, 540, Centro Londrina Parana´ Brazil Tel: +55 43 3371 6722 E-mail: [email protected]
Case Report TMJ Disorders
Congenital Frey’s syndrome associated with nontraumatic bilateral trifid mandibular condyle
J. Motta-Junior, T. G. Aita, C. L. Pereira-Stabile, G. A. V. Stabile Department of Oral and Maxillofacial Surgery, State University of Londrina, Londrina, Parana´, Brazil
J. Motta-Junior, T. G. Aita, C. L. Pereira-Stabile, G. A. V. Stabile: Congenital Frey’s syndrome associated with nontraumatic bilateral trifid mandibular condyle. Int. J. Oral Maxillofac. Surg. 2013; 42: 237–239. # 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. Congenital Frey’s syndrome and bilateral trifid mandibular condyle are two different entities. The occurrence of both together is rare and has not been reported in the literature. This article describes the case of a 17-year-old male patient who complained of bilateral warmness, flushing and sweating in the preauricular area after eating spicy and sour foods since childhood. He had no complaint related to the functions of the temporomandibular joint and had no history of facial trauma. The patient was treated with injections of botulinum toxin A and the signs and symptoms of Frey’s syndrome improved significantly.
Accepted for publication 22 June 2012 Available online 24 July 2012
Warmness, flushing and sweating in the preauricular area are signs and symptoms of Frey’s syndrome. This syndrome was first described in 1757, but its occurrence after auriculotemporal nerve damage was described in 1923 by the Polish neurologist, Łucja Frey.1 The syndrome can be associated with direct or indirect damage of the greater auricular nerve and/or the auriculotemporal nerve. An anatomically abnormal mandibular condyle is also unusual and can be associated with several situations, especially with previous trauma to the temporomandibular joint (TMJ). One of the abnormalities of the mandibular condyle is related to the number of condylar heads. The first report about this was published in 1941 by Hrdlicka.2 Only one specific case of trifid mandibular condyle associated with
There was no report of facial trauma. To support the diagnosis, orthopantomography was performed and trifid condyle heads were observed on both sides (Fig. 1). Considering the radiographic findings, a computed tomography (CT) of the mandible was performed. Three dimensional (3D) reconstruction of this examination revealed the trifid anatomy of both condyle heads and their relationship with the joint cavities (Fig. 2). Following the diagnostic imaging, Minor’s test was performed. A solution containing iodine and ethanol was applied to the preauricular area on both sides. When it was dry, corn starch powder was applied to the same areas. The patient was asked to eat a sour candy and after 30 s the preauricular areas tested positive with a bluish-black colour change because of the
0901-5027/020237 + 03 $36.00/0
a history of previous facial trauma has been reported3 and the congenital occurrence of a trifid condyle is uncommon. The aim of this paper is to report a case of congenital Frey’s syndrome associated with bilateral trifid mandibular condyle and to describe the treatment for this patient. Case report
A 17-year-old man was referred to the authors by his general dentist. His medical history was noncontributory. His complaints were bilateral warmness, flushing and sweating of the preauricular area after eating spicy and sour foods since childhood. His daily routine was particularly disturbed by this, mainly when eating in public. There was no history of the use of medication by his mother during pregnancy and his birth was reported as uneventful.
# 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
238
Motta-Junior et al.
Fig. 1. Orthopantomography displays the trifid condyle heads on both sides (arrows).
iodine–starch reaction, sweat secretion also increased (Fig. 3). For an exact determination of the hyperaesthetic regions, flushing and sweating areas were mapped and divided into 9 fields of 2 cm2 on each side. Based on the patient’s history and clinical, radiographic, 3D CT reconstruction and Minor’s test, the diagnosis of Frey’s syndrome associated with nontraumatic bilateral trifid mandibular condyle was suggested. The patient was treated conservatively with subcutaneous injection of botulinum toxin A (BTA) 25 U/0.1 ml in the centre of each field for treatment of the Frey’s syndrome. After 3 days, Minor’s test was performed again to confirm the effect of BTA; some remaining areas of sweating were observed. The patient had
no complaints after the application of BTA and has been followed-up for 2 years. Discussion
Trifid or bifid mandibular condyles are uncommon anomalies. There are two theories about this deformity. Walker4 sectioned and dislocated the condyle of monkeys and was able to show development of a new condylar head, providing evidence that fracture can cause new condyle formation. Blackwood5 advocated the theory of embryological malformation. According to this theory, a septum vascular fibre in the condylar cartilage is developed when the foetus is about 20 weeks old. After birth, in the ninth week
of life, the septum disappears and new bone formation on the condyle begins. If this septum remains, abnormal ossification can occur and cause bifid/trifid condyle development. In the present case report, the origin of the trifid condyles is probably related to embryological changes, because both condyles were similarly affected and according to the parents there was no history of facial trauma or surgery. Frey’s syndrome usually occurs in patients who have undergone submandibular or parotid salivary gland surgery6 because of the manipulation of the auriculotemporal nerve and the great auricular nerve. Another cause of this syndrome can be mandibular condyle fracture, due to the intimate anatomical relationship of the auriculotemporal nerve with the capsule of the TMJ. The most accepted theory to explain Frey’s syndrome is the theory of aberrant regeneration.7 The misdirected regeneration of parasympathetic fibres of the auriculotemporal nerve after facial trauma promotes simultaneous activation of parotid and sweat glands by acetylcholine. Another possible source for this aberrant innervation is direct contact of some great auricular nerve fibres with the auriculotemporal nerve.8 Regardless of the causes of the condyle abnormality or Frey’s syndrome, the objective of treatment must be focused on the signs and symptoms, which include
Fig. 2. 3D reconstruction showing the trifid mandible condyle: right (A) and left (B) with medial, lateral and posterior condylar heads.
Fig. 3. Positive Minor’s test. Sweating on left (A) and right (B) preauricular areas.
Congenital Frey’s syndrome and bilateral trifid mandibular condyle sweating, flushing and warmness of the cheek, retroauricular and temporal regions after eating.9 Options for treatment are surgery with skin excision and interposition of fascia lata or muscle flaps8; systemic or topical drugs with anticholinergic or antihidrotic effects; and intradermal injection of BTA.10 BTA has been shown to be effective in treating Frey’s syndrome treatment and after some applications the sweat glands of the skin can undergo atrophy. BTA prevents the release of acetylcholine in the synaptic gap of the nerves to the salivary and sweat glands. Although its action is not prolonged and its effect is temporary, it was the option of choice for treatment, because of its proven benefits.10 Besides being less invasive than surgical treatment, the costs and infection risks are diminished in BTA treatment. The patient’s age and previous treatment must also be considered when choosing between BTA and surgical treatment. This case has demonstrated that bilateral Frey’s syndrome and trifid condyle can occur simultaneously and have similar embryologic origin. Surgeons should be aware that these alterations can occur spontaneously and not only after surgery or trauma. In such cases the treatment given must consider the signs and symp-
toms and focus on promoting the patient’s wellbeing. Funding
None. Competing interests
None declared. Ethical approval
Not required. References 1. Jacobsen N, Hopkins C. The bullet that hit a nerve: the history of Lucja Frey and her syndrome. J Laryngol Otol 2006;120: 178–80. 2. Hrdlicka A. Lower jaw: double condyles. Am J Phys Anthropol 1941;28:75–89. 3. Artvinli LB, Kansu O. Trifid mandibular condyle: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;95:251–4. 4. Walker RV. Traumatic mandibular condylar fracture dislocations. Effect on growth in the Macaca rhesus monkey. Am J Surg 1960; 100:850–63.
239
5. Blackwood HJ. The double-headed mandibular condyle. Am J Phys Anthropol 1957;15:1–8. 6. Clayman MA, Clayman SM, Seagle MB. A review of the surgical and medical treatment of Frey syndrome. Ann Plast Surg 2006;57:581–4. 7. Gardner WJ, McCubbin JW. Auriculotemporal syndrome; gustatory sweating due to misdirection of regenerated nerve fibers. J Am Med Assoc 1956;160:272–7. 8. Laage-Hellman JE. Treatment of gustatory sweating and flushing. Acta Otolaryngol 1958;49:132–43. 9. Laskawi R, Ellies M, Rodel R, Schoenebeck C. Gustatory sweating: clinical implications and etiologic aspects. J Oral Maxillofac Surg 1999;57:642–8. [discussion 648–649]. 10. von Lindern JJ, Niederhagen B, Berge S, Reich RH. Treatment of Frey’s syndrome with type A botulinum toxin: case report. J Oral Maxillofac Surg 2000;58:1411–4.
Address: Joel Motta Junior Clı´nica Odontolo´gica Universita´ria Rua Pernambuco, 540, Centro Londrina Parana´ Brazil Tel: +55 43 3371 6722 E-mail: [email protected]