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Accessory auricles affecting the tragus and cheek occurring with cervical chondrocutaneous branchial remnants: A case report
JPRAS Open 6 (2015) 20e24
Contents lists available at ScienceDirect
JPRAS Open journal homepage: http://www.journals.elsevier.com/ jpras-open
Case report
Accessory auricles affecting the tragus and cheek occurring with cervical chondrocutaneous branchial remnants: A case report T. Kono*, H. Ro, N. Murakami, M. Kochi, T. Kuroki, Y. Tosa, S. Yoshimoto Department of Plastic and Reconstructive Surgery, Showa University School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo, 142-8666, Japan
a r t i c l e i n f o
a b s t r a c t
Article history: Received 29 May 2015 Accepted 7 September 2015 Available online 25 September 2015
Accessory auricles are protruding skin lesions commonly affecting the tragus, preauricular region, and cheeks. Similar lesions, termed cervical chrondrocutaneous branchial remnants, may occur in the neck; however, they are thought to have a differing development origin to accessory auricles. Accessory auricles affecting the face are believed to be derived from the first branchial arch, whereas cervical chrondrocutaneous branchial remnants are believed to be derived from the second branchial arch and more caudal-related structures. Cervical chrondrocutaneous branchial remnants occur at a frequency lower than accessory auricles affecting the face, with a very rare co-occurrence. Here, we report the case of a 6month-old girl with 4 congenital chondroid masses at 3 distinct sites: the right tragus, right cheek, and bilateral cervical regions. All masses were excised and diagnosed as accessory auricles and cervical chrondrocutaneous branchial remnants following histopathological analysis. The patient had no other coexisting conditions. We conducted a literature search and found only 5 previously reported Japanese cases with co-occurrence of cervical chrondrocutaneous branchial remnants and accessory auricles affecting the face. To our knowledge, this study is the first to present a case with the coexistence of cervical chrondrocutaneous branchial remnants and accessory auricles affecting the tragus and cheek. We believe the occurrence of cervical chrondrocutaneous branchial remnants in this case was not associated with the occurrence of accessory auricles affecting the tragus and cheek. However, an
Keywords: Accessory auricle Cervical auricle Cervical chrondrocutaneous branchial remnants Preauricular region Skin tag
* Corresponding author. Tel.: þ81 (0)3 3784 8548; fax: þ81 (0)3 3784 9183. E-mail address: [email protected] (T. Kono). http://dx.doi.org/10.1016/j.jpra.2015.09.002 2352-5878/© 2015 The Authors. Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and Aesthetic Surgeons. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
T. Kono et al. / JPRAS Open 6 (2015) 20e24
21
association cannot be completely ruled out as there are cases suggesting an association between the occurrence of cervical chrondrocutaneous branchial remnants and the development of auricles; the mechanisms underlying this development remains unelucidated. Therefore, further reports and studies are required. © 2015 The Authors. Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and Aesthetic Surgeons. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction Accessory auricles are a congenital disorder that may be observed during routine examination. Single or multiple accessory auricles may occur along a line connecting the tragus and oral commissure, mainly at the tragus, preauricular region, and cheek. Similar lesions in the neck have been termed “cervical auricles” or “cervical skin tags.” As cervical auricles and accessory auricles of the preauricular region developmentally differ, the term “cervical chrondrocutaneous branchial remnants (CCBRs)” has been proposed for these abnormalities.1,2 Only a few reports describe these lesions as benign and that they cause no functional impairment; they are generally resected solely for cosmetic reasons, with majority of them comprising cartilage tissues on histological examination. CCBRs is reported to occur at a lower frequency than accessory auricles affecting the face,3 and coexistence of both phenomena are believed to be very rare. However, other malformations are frequently observed in cases with CCBRs.2,4 Here, we report a case with coexisting bilateral CCBRs and accessory auricles affecting the tragus and cheek, with no other malformations. We conducted a brief literature review of similar previously reported cases. Case report A 6-month-old girl presented with the chief complaint of protruding lesions affecting the right tragus, right cheek, and bilateral cervical regions that had been present since birth. She presented to our hospital with her parents for resection of the lesions. Her birth history included an uneventful fullterm pregnancy, uneventful delivery, and no other birth defects. Family and medical histories were non-contributory. Findings at initial examination revealed 4 skin masses at 3 sites in the facial and cervical regions (Figure 1). There was no other congenital malformation. Lesional characteristics are presented in Table 1. Lesions were resected under general anesthesia. At each site, the skin was resected in a spindle shape around the center of the mass before excision of the mass and associated subcutaneous cartilage. The lesion affecting the right cheek was intraoperatively observed to comprise firm, cord-like chondroid tissues continuing from subcutaneous areas toward the tragus. The attachment site could not be identified; the lesion was resected midcourse (Figure 2). Histopathological examination revealed that resected accessory auricles and CCBRs comprised cartilage tissues, extending from the dermis into the hypodermis. Cartilage extracellular matrix was found to be rich in elastic fibers. These findings suggest that the resected cartilage was elastic cartilage. Discussion The predilection site of accessory auricles affecting the face is along a line connecting the tragus and oral commissure. This line closely coincides with the junction of the maxillary and mandibular prominences, which are derived from the first branchial arch. This area is derived from the first branchial arch superior to a line connecting the orifice of the external auditory meatus and the superior
22
T. Kono et al. / JPRAS Open 6 (2015) 20e24
Figure 1. Photograph demonstrating 4 skin masses at 3 sites in the facial and cervical region sites.
border of the hyoid bone (first branchial cleft). The majority of CCBRs are located in the area over the lower one-third of the sternocleidomastoid; this area is derived from the second branchial arch and more caudal-related structures and separated from the predilection sites for accessory auricles of the cheek by the first branchial cleft. Brownstein et al5 speculated that accessory auricles affecting the face are derived from the first branchial arch as the majority of them are located a line connecting the tragus and oral commissure. Accessory auricles may occur in first branchial arch syndrome; the tragus is derived from the first branchial arch. Mukai et al4 investigated auricle development and proposed that accessory auricles in the preauricular region and cheek are generally derived from the first branchial arch and that CCBRs are likely derived from the second branchial arch and more caudal structures as the sites and associated CCBRs conditions differ from that of accessory auricles. The reported locations and histological findings
Table 1 Lesion characteristics. Site
Form
Size
Histopathological examination
Right tragus Right cheek
Oval and elastic Pedunculated, club-shaped, and elastic Pedunculated, club-shaped, and elastic Pedunculated, club-shaped, and elastic
6 3 3 mm 10 10 13 mm
Elastic cartilage Elastic cartilage
13 13 15 mm
Elastic cartilage
10 10 13 mm
Elastic cartilage
Right cervical region (inferior region of the right sternocleidomastoid) Left cervical region (inferior region of the left sternocleidomastoid)
T. Kono et al. / JPRAS Open 6 (2015) 20e24
23
Figure 2. Intraoperative photograph of accessory auricle on the right cheek.
of accessory auricles in previous studies are consistent with those of the accessory auricles affecting the tragus and cheek in our case. Thus, the aforementioned, widely accepted explanation for accessory auricles occurrence is believed to be applicable in our case. Many accessory auricles affecting the cheek co-occur with accessory auricles of the periauricular region. Sakai et al6 examined 10 cases of accessory auricles affecting the cheek area from past reports in addition to 2 of their own cases. Eleven of the 12 cases were also found to have accessory auricles in the periauricular region. However, only a few cases had accessory auricles affecting the face co-occurring with CCBRs. Our literature search revealed that such cases are very rare, with only 5 previously reported cases (Table 2). The above findings suggest the accessory auricles affecting the tragus and cheek in our case were derived from the first branchial arch according to the widely accepted predominant theory. There is possibly a strong association between accessory auricles occurrences affecting the tragus and cheek. In Table 2 Cases of coexisting facial accessory auricles and chrondrocutaneous branchial remnants. Case Authors (year of publication)
Age (years) Sex Follow-up Combination of period accessory auricles and CCBRs (cases with one or the other)
1
Asano et al7 (1982)
2
Shinbashi et al8 14 years (1984)
1 case (156 cases) Unknown
3
Hayakawa et al9 (1986)
Unknown
1 case
4
Mukai et al4 (2001)
23 years
1 case (498 cases) 3
5
Sunohara et al3 (2013)
11 years
1 case (258 cases) Unknown
7 years
1 case (17 cases with CCBRs)
2
15
Site
Associated anomalies
Female
None
Female
Bilateral microtia, left external auditory meatus atresia None
Right side of the face, left cervical area Unknown Both sides of the face, unilateral cervical area
Left preauricular area, right cervical area Male Left side of the face, Bilateral congenital left cervical area aural fistulas, right cupped ear Unknown Unknown Unknown
24
T. Kono et al. / JPRAS Open 6 (2015) 20e24
contrast, CCBRs are believed to be derived from other branchial arches; their occurrence is unlikely to be developmentally associated with accessory auricles occurrence. However, the aforementioned association in the periauricular region cannot be conclusively ruled out. During development, auricles are formed by the fusion of the first, second, and third auricular hillocks, derived from the first branchial arch, and the fourth, fifth, and sixth auricular hillocks, derived from the second branchial arch. The 6 auricular hillocks first develop in an area that subsequently develops into the cervical region before migration to the lateral aspects of the head as the mandible and teeth develop and then ascend to the eye level. Thus, branchial arches are believed to be the embryological structures from which auricles develop. Accessory auricles in the periauricular region and CCBRs form along the developmental path of auricles, i.e., accessory auricles and CCBRs may develop from the first and second branchial arches from which the 6 auricular hillocks are derived. CCBRs have been reported to be continuous with auricles,10 suggesting a developmental association between CCBRs and accessory auricles in the periauricular region cannot be conclusively ruled out. Conclusion We report a case with coexisting CCBRs and accessory auricles affecting the tragus and cheek. To our knowledge, there have been no similar previously reported cases. It is highly possible that there is an association between the development of accessory auricles affecting the tragus and cheek based on common sites, frequency of their coexistence, and developmental course. However, an association between CCBRs and accessory auricle of the cheek is believed to be unlikely. In our case, we believe CCBRs coincidentally occurred with accessory auricles of the tragus and cheek. However, there are previously reported cases suggesting a developmental association between CCBRs and accessory auricles. Further accumulation of case reports is required to completely elucidate developmental mechanisms underlying the formation of accessory auricles. Funding None. Conflicts of interest None declared. Ethical approval Not required. References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10.
Nakamura J. Jpn J Plastic Reconstr Surg. 1991;34:445e453. Atlan G, Egerszegi EP, Brochu P, Caouette-Laberge L, Bortoluzzi P. Plast Reconstr Surg. 1997;100:32e39. Sunohara M. J Jpn Soc Plast Reconstr Surg. 2013;33:77e83. Mukai R. J Jpn Soc Plast Reconstr Surg. 2001;21:542e547. Brownstein MH, Wanger N, Helwig EB. Arch Dermatol. 1971;104:625e631. Sakai K. Rinsho Derma (Tokyo). 1991;33:1313e1317. Asano T. J Jpn Soc Plast Reconstr Surg. 1982;2:60e66. Shinbashi T. Jpn J Plast Reconstr Surg. 1984;27:456e463. Hayakawa K. Rinsho Derma (Tokyo). 1986;28:946e947. Udagawa A. Jpn J Plast Reconstr Surg. 1985;28:509e513.
Contents lists available at ScienceDirect
JPRAS Open journal homepage: http://www.journals.elsevier.com/ jpras-open
Case report
Accessory auricles affecting the tragus and cheek occurring with cervical chondrocutaneous branchial remnants: A case report T. Kono*, H. Ro, N. Murakami, M. Kochi, T. Kuroki, Y. Tosa, S. Yoshimoto Department of Plastic and Reconstructive Surgery, Showa University School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo, 142-8666, Japan
a r t i c l e i n f o
a b s t r a c t
Article history: Received 29 May 2015 Accepted 7 September 2015 Available online 25 September 2015
Accessory auricles are protruding skin lesions commonly affecting the tragus, preauricular region, and cheeks. Similar lesions, termed cervical chrondrocutaneous branchial remnants, may occur in the neck; however, they are thought to have a differing development origin to accessory auricles. Accessory auricles affecting the face are believed to be derived from the first branchial arch, whereas cervical chrondrocutaneous branchial remnants are believed to be derived from the second branchial arch and more caudal-related structures. Cervical chrondrocutaneous branchial remnants occur at a frequency lower than accessory auricles affecting the face, with a very rare co-occurrence. Here, we report the case of a 6month-old girl with 4 congenital chondroid masses at 3 distinct sites: the right tragus, right cheek, and bilateral cervical regions. All masses were excised and diagnosed as accessory auricles and cervical chrondrocutaneous branchial remnants following histopathological analysis. The patient had no other coexisting conditions. We conducted a literature search and found only 5 previously reported Japanese cases with co-occurrence of cervical chrondrocutaneous branchial remnants and accessory auricles affecting the face. To our knowledge, this study is the first to present a case with the coexistence of cervical chrondrocutaneous branchial remnants and accessory auricles affecting the tragus and cheek. We believe the occurrence of cervical chrondrocutaneous branchial remnants in this case was not associated with the occurrence of accessory auricles affecting the tragus and cheek. However, an
Keywords: Accessory auricle Cervical auricle Cervical chrondrocutaneous branchial remnants Preauricular region Skin tag
* Corresponding author. Tel.: þ81 (0)3 3784 8548; fax: þ81 (0)3 3784 9183. E-mail address: [email protected] (T. Kono). http://dx.doi.org/10.1016/j.jpra.2015.09.002 2352-5878/© 2015 The Authors. Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and Aesthetic Surgeons. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
T. Kono et al. / JPRAS Open 6 (2015) 20e24
21
association cannot be completely ruled out as there are cases suggesting an association between the occurrence of cervical chrondrocutaneous branchial remnants and the development of auricles; the mechanisms underlying this development remains unelucidated. Therefore, further reports and studies are required. © 2015 The Authors. Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and Aesthetic Surgeons. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).
Introduction Accessory auricles are a congenital disorder that may be observed during routine examination. Single or multiple accessory auricles may occur along a line connecting the tragus and oral commissure, mainly at the tragus, preauricular region, and cheek. Similar lesions in the neck have been termed “cervical auricles” or “cervical skin tags.” As cervical auricles and accessory auricles of the preauricular region developmentally differ, the term “cervical chrondrocutaneous branchial remnants (CCBRs)” has been proposed for these abnormalities.1,2 Only a few reports describe these lesions as benign and that they cause no functional impairment; they are generally resected solely for cosmetic reasons, with majority of them comprising cartilage tissues on histological examination. CCBRs is reported to occur at a lower frequency than accessory auricles affecting the face,3 and coexistence of both phenomena are believed to be very rare. However, other malformations are frequently observed in cases with CCBRs.2,4 Here, we report a case with coexisting bilateral CCBRs and accessory auricles affecting the tragus and cheek, with no other malformations. We conducted a brief literature review of similar previously reported cases. Case report A 6-month-old girl presented with the chief complaint of protruding lesions affecting the right tragus, right cheek, and bilateral cervical regions that had been present since birth. She presented to our hospital with her parents for resection of the lesions. Her birth history included an uneventful fullterm pregnancy, uneventful delivery, and no other birth defects. Family and medical histories were non-contributory. Findings at initial examination revealed 4 skin masses at 3 sites in the facial and cervical regions (Figure 1). There was no other congenital malformation. Lesional characteristics are presented in Table 1. Lesions were resected under general anesthesia. At each site, the skin was resected in a spindle shape around the center of the mass before excision of the mass and associated subcutaneous cartilage. The lesion affecting the right cheek was intraoperatively observed to comprise firm, cord-like chondroid tissues continuing from subcutaneous areas toward the tragus. The attachment site could not be identified; the lesion was resected midcourse (Figure 2). Histopathological examination revealed that resected accessory auricles and CCBRs comprised cartilage tissues, extending from the dermis into the hypodermis. Cartilage extracellular matrix was found to be rich in elastic fibers. These findings suggest that the resected cartilage was elastic cartilage. Discussion The predilection site of accessory auricles affecting the face is along a line connecting the tragus and oral commissure. This line closely coincides with the junction of the maxillary and mandibular prominences, which are derived from the first branchial arch. This area is derived from the first branchial arch superior to a line connecting the orifice of the external auditory meatus and the superior
22
T. Kono et al. / JPRAS Open 6 (2015) 20e24
Figure 1. Photograph demonstrating 4 skin masses at 3 sites in the facial and cervical region sites.
border of the hyoid bone (first branchial cleft). The majority of CCBRs are located in the area over the lower one-third of the sternocleidomastoid; this area is derived from the second branchial arch and more caudal-related structures and separated from the predilection sites for accessory auricles of the cheek by the first branchial cleft. Brownstein et al5 speculated that accessory auricles affecting the face are derived from the first branchial arch as the majority of them are located a line connecting the tragus and oral commissure. Accessory auricles may occur in first branchial arch syndrome; the tragus is derived from the first branchial arch. Mukai et al4 investigated auricle development and proposed that accessory auricles in the preauricular region and cheek are generally derived from the first branchial arch and that CCBRs are likely derived from the second branchial arch and more caudal structures as the sites and associated CCBRs conditions differ from that of accessory auricles. The reported locations and histological findings
Table 1 Lesion characteristics. Site
Form
Size
Histopathological examination
Right tragus Right cheek
Oval and elastic Pedunculated, club-shaped, and elastic Pedunculated, club-shaped, and elastic Pedunculated, club-shaped, and elastic
6 3 3 mm 10 10 13 mm
Elastic cartilage Elastic cartilage
13 13 15 mm
Elastic cartilage
10 10 13 mm
Elastic cartilage
Right cervical region (inferior region of the right sternocleidomastoid) Left cervical region (inferior region of the left sternocleidomastoid)
T. Kono et al. / JPRAS Open 6 (2015) 20e24
23
Figure 2. Intraoperative photograph of accessory auricle on the right cheek.
of accessory auricles in previous studies are consistent with those of the accessory auricles affecting the tragus and cheek in our case. Thus, the aforementioned, widely accepted explanation for accessory auricles occurrence is believed to be applicable in our case. Many accessory auricles affecting the cheek co-occur with accessory auricles of the periauricular region. Sakai et al6 examined 10 cases of accessory auricles affecting the cheek area from past reports in addition to 2 of their own cases. Eleven of the 12 cases were also found to have accessory auricles in the periauricular region. However, only a few cases had accessory auricles affecting the face co-occurring with CCBRs. Our literature search revealed that such cases are very rare, with only 5 previously reported cases (Table 2). The above findings suggest the accessory auricles affecting the tragus and cheek in our case were derived from the first branchial arch according to the widely accepted predominant theory. There is possibly a strong association between accessory auricles occurrences affecting the tragus and cheek. In Table 2 Cases of coexisting facial accessory auricles and chrondrocutaneous branchial remnants. Case Authors (year of publication)
Age (years) Sex Follow-up Combination of period accessory auricles and CCBRs (cases with one or the other)
1
Asano et al7 (1982)
2
Shinbashi et al8 14 years (1984)
1 case (156 cases) Unknown
3
Hayakawa et al9 (1986)
Unknown
1 case
4
Mukai et al4 (2001)
23 years
1 case (498 cases) 3
5
Sunohara et al3 (2013)
11 years
1 case (258 cases) Unknown
7 years
1 case (17 cases with CCBRs)
2
15
Site
Associated anomalies
Female
None
Female
Bilateral microtia, left external auditory meatus atresia None
Right side of the face, left cervical area Unknown Both sides of the face, unilateral cervical area
Left preauricular area, right cervical area Male Left side of the face, Bilateral congenital left cervical area aural fistulas, right cupped ear Unknown Unknown Unknown
24
T. Kono et al. / JPRAS Open 6 (2015) 20e24
contrast, CCBRs are believed to be derived from other branchial arches; their occurrence is unlikely to be developmentally associated with accessory auricles occurrence. However, the aforementioned association in the periauricular region cannot be conclusively ruled out. During development, auricles are formed by the fusion of the first, second, and third auricular hillocks, derived from the first branchial arch, and the fourth, fifth, and sixth auricular hillocks, derived from the second branchial arch. The 6 auricular hillocks first develop in an area that subsequently develops into the cervical region before migration to the lateral aspects of the head as the mandible and teeth develop and then ascend to the eye level. Thus, branchial arches are believed to be the embryological structures from which auricles develop. Accessory auricles in the periauricular region and CCBRs form along the developmental path of auricles, i.e., accessory auricles and CCBRs may develop from the first and second branchial arches from which the 6 auricular hillocks are derived. CCBRs have been reported to be continuous with auricles,10 suggesting a developmental association between CCBRs and accessory auricles in the periauricular region cannot be conclusively ruled out. Conclusion We report a case with coexisting CCBRs and accessory auricles affecting the tragus and cheek. To our knowledge, there have been no similar previously reported cases. It is highly possible that there is an association between the development of accessory auricles affecting the tragus and cheek based on common sites, frequency of their coexistence, and developmental course. However, an association between CCBRs and accessory auricle of the cheek is believed to be unlikely. In our case, we believe CCBRs coincidentally occurred with accessory auricles of the tragus and cheek. However, there are previously reported cases suggesting a developmental association between CCBRs and accessory auricles. Further accumulation of case reports is required to completely elucidate developmental mechanisms underlying the formation of accessory auricles. Funding None. Conflicts of interest None declared. Ethical approval Not required. References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10.
Nakamura J. Jpn J Plastic Reconstr Surg. 1991;34:445e453. Atlan G, Egerszegi EP, Brochu P, Caouette-Laberge L, Bortoluzzi P. Plast Reconstr Surg. 1997;100:32e39. Sunohara M. J Jpn Soc Plast Reconstr Surg. 2013;33:77e83. Mukai R. J Jpn Soc Plast Reconstr Surg. 2001;21:542e547. Brownstein MH, Wanger N, Helwig EB. Arch Dermatol. 1971;104:625e631. Sakai K. Rinsho Derma (Tokyo). 1991;33:1313e1317. Asano T. J Jpn Soc Plast Reconstr Surg. 1982;2:60e66. Shinbashi T. Jpn J Plast Reconstr Surg. 1984;27:456e463. Hayakawa K. Rinsho Derma (Tokyo). 1986;28:946e947. Udagawa A. Jpn J Plast Reconstr Surg. 1985;28:509e513.