- Email: [email protected]
Congenital cutaneous fistula at the sternoclavicular joint – Not a dermoid fistula but the remnant of the fourth branchial (pharyngeal) cleft ?
Accepted Manuscript Title: Congenital cutaneous fistula at the sternoclavicular joint- not a dermoid fistula but the remnant of the fourth branchial (pharyngeal) cleft?Author: Michinobu Ohno Yutaka Kanamori Kotaro Tomonaga Tatsuya Yamashita Misato Migita Toshiko Takezoe Toshihiko Watanabe Yasushi Fuchimoto Kentaro Matsuoka PII: DOI: Reference:
S0165-5876(15)00472-3 http://dx.doi.org/doi:10.1016/j.ijporl.2015.09.025 PEDOT 7772
To appear in:
International Journal of Pediatric Otorhinolaryngology
Received date: Revised date: Accepted date:
9-7-2015 18-9-2015 21-9-2015
Please cite this article as: M. Ohno, Y. Kanamori, K. Tomonaga, T. Yamashita, M. Migita, T. Takezoe, T. Watanabe, Y. Fuchimoto, K. Matsuoka, Congenital cutaneous fistula at the sternoclavicular joint- not a dermoid fistula but the remnant of the fourth branchial (pharyngeal) cleft?-, International Journal of Pediatric Otorhinolaryngology (2015), http://dx.doi.org/10.1016/j.ijporl.2015.09.025 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Congenital cutaneous fistula at the sternoclavicular joint - not a dermoid fistula but the remnant of the fourth branchial (pharyngeal) cleft ?-
Michinobu Ohno1, Yutaka Kanamori1, Kotaro Tomonaga1, Tatsuya Yamashita1, Misato
ip t
Migita1, Toshiko Takezoe1, Toshihiko Watanabe1, Yasushi Fuchimoto1, Kentaro
1 Division of Surgery, Department of Surgical Specialties
National Center for Child Health and Development
an
2-10-1 Okura Setagaya-ku Tokyo, 157-8535, Japan
us
2 Department of Pathology
cr
Matsuoka2
Correspondence to: Yutaka Kanamori, M.D.
M
Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, Tokyo, Japan
ed
2-10-1 Okura, Setagaya, Tokyo 157-8535, Japan
Runninghead:
pt
E-mail; [email protected], Tel; +81-3-3416-0181, Fax; +81-3-3416-2222
Ac ce
Cutaneous fistula originated from the fourth pharyngeal cleft remnant
Key words:
Cutaneous fistula, fourth branchial cleft, pyriform sinus fistula
Page 1 of 14
Abstract A fourth branchial pouch remnant is well known as a pyriform sinus fistula. However, there has been no report of a fistula composed of the complete remnant of the
ip t
fourth branchial apparatus. We experienced patients with a congenital lower neck cutaneous fistula which was thought to be the skin-side remnant of the fourth branchial cleft.
cr
Materials and methods: Seven children were referred to our hospital from 2009 to 2015 for the treatment of a cutaneous fistula situated near the sternoclavicular joint. All of
Clinical charts were retrospectively reviewed.
us
them were surgically resected and their pathological characteristics were examined.
an
Results: In six cases, the left side was affected. All cutaneous fistulas had a small skin orifice near the sternoclavicular joint and they were situated at the anterior edge of the sternocleidomastoid muscle. Abscess formation was seen in four cases. Surgical
M
resection was performed at the age of 6 months to 9 years. These fistulas ran deep into the subcutaneous tissue and had a blind end. Pathological examination showed that
ed
the epithelial layer was mainly composed of a stratified squamous epithelium. In two cases the epithelium was composed of ciliated columnar epithelium. Recurrence has not been observed in any of the cases.
pt
Conclusion: The seven cases had a common clinical feature and were a definite clinical entity. Judging from the characteristics of our cases and the previous literature, we
Ac ce
concluded that this lower neck cutaneous fistula was most likely a congenital skin-side remnant of the fourth branchial cleft.
Page 2 of 14
Introduction Most congenital lateral cervical fistulas, cysts, and sinuses are considered to derive from the first to fourth branchial (pharyngeal) arch and related parts. Up to 95% of
ip t
cases of branchial arch remnant had arisen from the second pharyngeal arch, while the remaining 5% had originated from the first, third or fourth arch(1). Fourth branchial
arch remnants are quite rare among all branchial arch anomaly(1,2) and they are now
cr
well known as a pyriform sinus fistula. A pyriform sinus fistula is a partial remnant of
the fourth or third branchial pouch and its clinical presentation has been reported(3-5).
us
However, a complete fistula of the fourth branchial pouch and cleft has never been reported.
an
We have experienced seven cases of a congenital lower neck cutaneous fistula situated at the anterior border of the sternocleidomastoid muscle and its cutaneous orifice was near the sternoclavicular joint. We describe details of the fistula and discuss
Ac ce
pt
ed
M
the origin of these fistulas.
Page 3 of 14
Patients and methods From 2009 to 2015, seven children underwent surgical resection of a congenital lower neck cutaneous fistula in our hospital. Records of these patients were reviewed along
ip t
with their clinical characteristics including patients’ age, gender, affected site, signs and
Ac ce
pt
ed
M
an
us
cr
symptoms, surgical findings and results of the pathological examination.
Page 4 of 14
Results This series included five boys and two girls (Table 1). The age at the time of surgical resection ranged from 6 months to 9 years (mean age: 2.3 years). The left side was
ip t
affected in six cases and the right side in only one case. All cases presented a congenital small pit on the skin of the lower neck that was noticed since birth. The pit was located near the sternoclavicular joint that was the anterior border of the sternocleidomastoid
cr
muscle and on the line of the clavicular bone (Fig. 1-a). Ultrasound examination was
performed in three patients and demonstrated that the cutaneous fistula ran along the
us
cephalic side of the clavicular bone and attenuated in the subcutaneous region. A contrast medium study was performed in one patient, and the course of the tract could
an
be visualized (Fig.1-b). Infection or abscess formation of the fistula was seen before the operation in four cases.
Surgical resection of the fistula was performed in all seven cases. All of the fistulas
M
penetrated the platysma muscle and ended in the subcutaneous tissue (Fig1-c). The mean length of the fistula was 13mm (range, 6mm to 22mm).
ed
Histopathological examination of the epithelial layer of the fistulas revealed that most cases had a stratified squamous epithelium (case 1-6) and four of them had an epidermal appendage such as a sebaceous gland, hair follicle or sweat gland (case 2-5)
pt
(Fig. 2-a). In three cases, the sinuses contained abundant keratinous material (case 4-6). In case 1 the epithelial layer was lined by the ciliated columnar epithelium in the
Ac ce
deep area of the fistula (Fig. 2-b). In case 7 the epithelial layer was mainly composed of the ciliated columnar epithelium and the inner lumen contained a mucinous fluid (Fig. 2-c). A salivary gland was detected around the ciliated columnar epithelium in case 1 (Fig. 2-d). None of the cases experienced clinical recurrence.
Page 5 of 14
Discussion The embryonic branchial apparatus is composed of five paired mesodermal arches separated by four pairs of endodermal and ectodermal invaginations. A branchial arch
ip t
fistula involves a branchial pouch and cleft, with rupture of the interposed branchial plate. A sinus tract may open to either the gut lumen or the skin(6). Among them, the
fourth branchial fistula is well known as a pyriform sinus fistula and it is derived from
cr
the remnant of the fourth branchial pouch. However, a complete continuous fistula of
the fourth branchial pouch and cleft has not been reported previously. The theoretical
us
entire tract of the fourth branchial remnant is very complicated. Liston(7) described the theoretical full course of a fourth branchial fistula (Fig. 3-a). It has two turning points
an
and runs from the apex of the pyriform sinus to a skin opening along a long course. An interesting fact is that the remnant fistula of the fourth branchial pouch is now well known as a pyriform sinus fistula, but the fourth branchial cleft fistula, in other
M
words the skin side of the fourth branchial remnant fistula, is not well known. A search of the English literature revealed that there is no definite report of the disease,
ed
although Liston showed its possible existence in the figure of his paper (Fig. 3-a). Here we reported seven cases of lower neck fistula. Features of lower neck fistula were as follows: 1) Most fistulas were situated on the left side since birth. 2) The site of
pt
the fistula orifice was at exactly the same point: near the sternoclavicular joint. The fistula orifice was situated at the anterior border of the sternocleidomastoid muscle and
Ac ce
on the line of the clavicular bone. 3) All of them had a short tract and a blind end in subcutaneous tissue. 4) Symptoms were seen in childhood and infection of the tract often occurred. 5) Their lining epithelium was mainly of ectodermal origin, consisting of a stratified squamous epithelium with skin adnexa. However, some fistulas contained ciliated columnar epithelium of endodermal origin. Considering these characteristics, we concluded that these fistulas should be viewed as one disease entity of congenital neck fistula. Four papers previously reported a very similar cutaneous fistula and all of them were published in dermatology journal(8-11). Those reports concluded that the fistulas were derived from ectodermal origin and they were designated as a dermoid fistula. We conjecture that the authors who insisted that the fistula was a dermoid fistula may not
Page 6 of 14
have kept the existence of the fourth branchial cleft remnant in mind because they were all dermatologists and may not have been familiar with branchial anomalies. The clinical characteristics of our case series were similar to those of the previously-reported cases(8-11). However, our cases differed from a dermoid fistula with regard to the
ip t
following points: 1) A dermoid fistula is usually situated on the midline of the body, and our case and the previously-reported cases were not situated on the midline. 2) The skin orifice of the fistulas was at the sternoclavicular joint which was near the anterior
cr
border of the sternocleidomastoid muscle. The second branchial remnant fistula was situated on the same border but usually at a more cranial side. Liston clearly stated
us
that the fourth branchial cleft remnant fistula theoretically opens on the anterior border of the sternocleidomastoid muscle. The textbook of human development by
an
Moore and Persaud(6) also suggested a possible fistula at the sternoclavicular joint (Fig. 3-b). 3) Among all reported cases(8-11) as well as our cases, the lesions were predominantly situated on the left side, which strongly suggested that the fistula was
M
related to the fourth branchial remnant fistula. 3) The lined epithelium in our cases 1 and 7 included a ciliated columnar epithelium (the epithelium was derived from
ed
endodermal origin). In case 1, a salivary gland was detected around the fistula. A dermoid fistula does not have these characteristics, and the branchial remnant fistula often has endodermal epithelium and a salivary gland is derived from the branchial
pt
arch. These points suggest that the cutaneous fistula reported herein is a fourth branchial cleft remnant and do not support that the fistula is a dermoid fistula. Of
Ac ce
course we cannot completely deny the possibility that these fistula is the second branchial cleft remnant but from its laterality and site we insist these fistula is more likely to be a fourth branchial cleft remnant.
Page 7 of 14
Conclusion We conclude that the seven cases of congenital lower neck fistula presented in this manuscript represent one disease entity, and judging from several clinical
ip t
characteristics, it may more likely be a fourth branchial cleft remnant fistula than a second branchial cleft remnant. Several similar cases had been reported in the past, but those authors considered the fistula to be a dermoid fistula. The present report is the
Ac ce
pt
ed
M
an
us
cr
first to insist that the fistula is a fourth branchial cleft remnant fistula.
Page 8 of 14
References 1.
Cote DN, Gianoli GJ. Fourth branchial cleft cysts. Otolaryngol Head Neck Surg 1996; 114: 95-97 Choi SS, Zalzal GH. Branchial anomalies: a review of 52 cases. Laryngoscope 1995; 105: 909-913
3.
Godin MS, Kearns DB, Pransky SM, et al. Fourth Branchial pouch sinus: principles
cr
of diagnosis and management. Laryngoscope 1990; 100: 174-178 4.
ip t
2.
Verret DJ, McClay J, Murray A, et al. Endoscopic cauterization of fourth branchial
5.
us
cleft sinus tract. Arch Otolaryngol Head Neck Surg 2004; 130: 465-468
Nicoucar K, Giger R, Pope Jr HG, et al. Management of congenital fourth branchial
an
arch anomalies: a review and analysis of published cases. J Pediatr Surg 2009; 44: 1432-1439
Moore KL and Persaud TVN. The pharyngeal organs. The Developing Human, 7th ed., Saunders, Philadelphia, 2007.
M
6.
Liston SL. Fourth branchial fistula. Otolaryngol Head Neck Surg 1981; 89: 520-522
8.
Matsunaga W, Ishihara T, Yasuno K. Congenital dermoid fistula of the anterior
ed
7.
chest region. Nishinihon J Dermatol 1994; 56: 34-39 9.
Muto J, Mori N, Konohara I, et al. Congenital dermoid fistula of the anterior chest
pt
region. Clin Exp Dermatol 2004; 29: 96-97 10. Numajiri T, Nishino K, Uenaka M, et al. Congenital dermoid fistula of the anterior
Ac ce
chest region. Acta Derm Venereol 2008; 88:538-539
11. Miyamoto T, Hosoda Y, Fujimoto Y, et al. Congenital skin fistula with sternal cleft. Br J Dermatol 1995; 132: 492-494
Page 9 of 14
Figure legends Figure 1. (a) The skin orifice of the fistula is situated near the sternoclavicular joint and
ip t
subcutaneous inflammation is seen around the fistula in this case. (case 1) (b) Preoperative contrast medium study shows that the fistula (arrow) runs toward the head of the left clavicular bone (arrowhead). (case 6)
cr
(c) Operative findings. The fistula is dilated like a cyst and then attenuates in the
us
subcutaneous region. Finally, the fistula ends blindly there. (case 6)
Figure 2. Histopathological examination of the fistulas (a, 100X; b, 100X; c, 100X; d,
an
40X), HE staining
(a) In most cases, the fistula was lined by stratified squamous epithelium. (case 1) (b) In case 7 the epithelium was mainly composed of ciliated columnar epithelium. The
M
internal lumen contained a mucinous fluid.
(c) In case 1 the inner layer of the fistula changed to ciliated columnar epithelium in the
ed
deeper area.
in case 1.
Figure 3.
pt
(d) A salivary gland was detected around the ciliated columnar epithelium of the fistula
Ac ce
(a) The theoretical entire course of the fourth branchial fistula. The figure also suggests the existence of a skin-side fistula (a figure cited from reference 7). The arrow shows a skin-sided fistula of the fourth branchial cleft. (b) Some possible opening sites of the branchial fistula or sinus are shown in the textbook of Moore and Persaud6). The arrow shows an external opening site that might have originated from the fourth branchial fistula near the sternoclavicular joint (a figure cited from reference 6).
Page 10 of 14
Table(s)
Table 1 Profile and characters of the seven patients Location
Length of fistula (mm)
Operation
Pathological findings
Stratified squamous epithelium, Fistulectomy ciliated columnar epitheium, salivary gland
－
Left
Female At birth
9y3m
－
Right
Sternoclavicular 6 joint
Fistulectomy
Stratified squamous epithelium, epidermal appendage
Male
2y7m
＋
Left
Sternoclavicular 11 joint
Fistulectomy
Stratified squamous epithelium, epidermal appendage
Left
Sternoclavicular 5 joint
Stratified squamous epithelium, Fistulectomy epidermal appendage, keratinous material
Left
Sternoclavicular 22 joint
Stratified squamous epithelium, Fistulectomy epidermal appendage, keratinous material
Left
Sternoclavicular 17 joint
Fistulectomy
Left
Sternoclavicular 15 joint
Fistulectomy Ciliated columnar epithelium
Female At birth
7m
＋
Male
At birth
6m
－
6
Male
At birth
1y5m
＋
7
Male
At birth
1y4m
Ac ce
pt
5
＋
us
At birth
an
3
M
2
At birth
ed
Male
cr
7m
Sternoclavicular 16 joint
1
4
Affected Side
ip t
Case Gender Noticed age Treated age Infection
Stratified squamous epithelium, keratinous material
Page 11 of 14
us
cr
i
Figure1
Ac
ce pt
ed
M an
Figure 1
(a)
(b)
(c) Page 12 of 14
ce pt
(a)
ed
M an
us
cr
i
Figure 2
(b)
Ac
Figure(s)
(c)
(d)
Page 13 of 14
i
Figure 3
Ac
ce pt
ed
M an
us
cr
Figure 3
(a)
(b)
Page 14 of 14
S0165-5876(15)00472-3 http://dx.doi.org/doi:10.1016/j.ijporl.2015.09.025 PEDOT 7772
To appear in:
International Journal of Pediatric Otorhinolaryngology
Received date: Revised date: Accepted date:
9-7-2015 18-9-2015 21-9-2015
Please cite this article as: M. Ohno, Y. Kanamori, K. Tomonaga, T. Yamashita, M. Migita, T. Takezoe, T. Watanabe, Y. Fuchimoto, K. Matsuoka, Congenital cutaneous fistula at the sternoclavicular joint- not a dermoid fistula but the remnant of the fourth branchial (pharyngeal) cleft?-, International Journal of Pediatric Otorhinolaryngology (2015), http://dx.doi.org/10.1016/j.ijporl.2015.09.025 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Congenital cutaneous fistula at the sternoclavicular joint - not a dermoid fistula but the remnant of the fourth branchial (pharyngeal) cleft ?-
Michinobu Ohno1, Yutaka Kanamori1, Kotaro Tomonaga1, Tatsuya Yamashita1, Misato
ip t
Migita1, Toshiko Takezoe1, Toshihiko Watanabe1, Yasushi Fuchimoto1, Kentaro
1 Division of Surgery, Department of Surgical Specialties
National Center for Child Health and Development
an
2-10-1 Okura Setagaya-ku Tokyo, 157-8535, Japan
us
2 Department of Pathology
cr
Matsuoka2
Correspondence to: Yutaka Kanamori, M.D.
M
Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, Tokyo, Japan
ed
2-10-1 Okura, Setagaya, Tokyo 157-8535, Japan
Runninghead:
pt
E-mail; [email protected], Tel; +81-3-3416-0181, Fax; +81-3-3416-2222
Ac ce
Cutaneous fistula originated from the fourth pharyngeal cleft remnant
Key words:
Cutaneous fistula, fourth branchial cleft, pyriform sinus fistula
Page 1 of 14
Abstract A fourth branchial pouch remnant is well known as a pyriform sinus fistula. However, there has been no report of a fistula composed of the complete remnant of the
ip t
fourth branchial apparatus. We experienced patients with a congenital lower neck cutaneous fistula which was thought to be the skin-side remnant of the fourth branchial cleft.
cr
Materials and methods: Seven children were referred to our hospital from 2009 to 2015 for the treatment of a cutaneous fistula situated near the sternoclavicular joint. All of
Clinical charts were retrospectively reviewed.
us
them were surgically resected and their pathological characteristics were examined.
an
Results: In six cases, the left side was affected. All cutaneous fistulas had a small skin orifice near the sternoclavicular joint and they were situated at the anterior edge of the sternocleidomastoid muscle. Abscess formation was seen in four cases. Surgical
M
resection was performed at the age of 6 months to 9 years. These fistulas ran deep into the subcutaneous tissue and had a blind end. Pathological examination showed that
ed
the epithelial layer was mainly composed of a stratified squamous epithelium. In two cases the epithelium was composed of ciliated columnar epithelium. Recurrence has not been observed in any of the cases.
pt
Conclusion: The seven cases had a common clinical feature and were a definite clinical entity. Judging from the characteristics of our cases and the previous literature, we
Ac ce
concluded that this lower neck cutaneous fistula was most likely a congenital skin-side remnant of the fourth branchial cleft.
Page 2 of 14
Introduction Most congenital lateral cervical fistulas, cysts, and sinuses are considered to derive from the first to fourth branchial (pharyngeal) arch and related parts. Up to 95% of
ip t
cases of branchial arch remnant had arisen from the second pharyngeal arch, while the remaining 5% had originated from the first, third or fourth arch(1). Fourth branchial
arch remnants are quite rare among all branchial arch anomaly(1,2) and they are now
cr
well known as a pyriform sinus fistula. A pyriform sinus fistula is a partial remnant of
the fourth or third branchial pouch and its clinical presentation has been reported(3-5).
us
However, a complete fistula of the fourth branchial pouch and cleft has never been reported.
an
We have experienced seven cases of a congenital lower neck cutaneous fistula situated at the anterior border of the sternocleidomastoid muscle and its cutaneous orifice was near the sternoclavicular joint. We describe details of the fistula and discuss
Ac ce
pt
ed
M
the origin of these fistulas.
Page 3 of 14
Patients and methods From 2009 to 2015, seven children underwent surgical resection of a congenital lower neck cutaneous fistula in our hospital. Records of these patients were reviewed along
ip t
with their clinical characteristics including patients’ age, gender, affected site, signs and
Ac ce
pt
ed
M
an
us
cr
symptoms, surgical findings and results of the pathological examination.
Page 4 of 14
Results This series included five boys and two girls (Table 1). The age at the time of surgical resection ranged from 6 months to 9 years (mean age: 2.3 years). The left side was
ip t
affected in six cases and the right side in only one case. All cases presented a congenital small pit on the skin of the lower neck that was noticed since birth. The pit was located near the sternoclavicular joint that was the anterior border of the sternocleidomastoid
cr
muscle and on the line of the clavicular bone (Fig. 1-a). Ultrasound examination was
performed in three patients and demonstrated that the cutaneous fistula ran along the
us
cephalic side of the clavicular bone and attenuated in the subcutaneous region. A contrast medium study was performed in one patient, and the course of the tract could
an
be visualized (Fig.1-b). Infection or abscess formation of the fistula was seen before the operation in four cases.
Surgical resection of the fistula was performed in all seven cases. All of the fistulas
M
penetrated the platysma muscle and ended in the subcutaneous tissue (Fig1-c). The mean length of the fistula was 13mm (range, 6mm to 22mm).
ed
Histopathological examination of the epithelial layer of the fistulas revealed that most cases had a stratified squamous epithelium (case 1-6) and four of them had an epidermal appendage such as a sebaceous gland, hair follicle or sweat gland (case 2-5)
pt
(Fig. 2-a). In three cases, the sinuses contained abundant keratinous material (case 4-6). In case 1 the epithelial layer was lined by the ciliated columnar epithelium in the
Ac ce
deep area of the fistula (Fig. 2-b). In case 7 the epithelial layer was mainly composed of the ciliated columnar epithelium and the inner lumen contained a mucinous fluid (Fig. 2-c). A salivary gland was detected around the ciliated columnar epithelium in case 1 (Fig. 2-d). None of the cases experienced clinical recurrence.
Page 5 of 14
Discussion The embryonic branchial apparatus is composed of five paired mesodermal arches separated by four pairs of endodermal and ectodermal invaginations. A branchial arch
ip t
fistula involves a branchial pouch and cleft, with rupture of the interposed branchial plate. A sinus tract may open to either the gut lumen or the skin(6). Among them, the
fourth branchial fistula is well known as a pyriform sinus fistula and it is derived from
cr
the remnant of the fourth branchial pouch. However, a complete continuous fistula of
the fourth branchial pouch and cleft has not been reported previously. The theoretical
us
entire tract of the fourth branchial remnant is very complicated. Liston(7) described the theoretical full course of a fourth branchial fistula (Fig. 3-a). It has two turning points
an
and runs from the apex of the pyriform sinus to a skin opening along a long course. An interesting fact is that the remnant fistula of the fourth branchial pouch is now well known as a pyriform sinus fistula, but the fourth branchial cleft fistula, in other
M
words the skin side of the fourth branchial remnant fistula, is not well known. A search of the English literature revealed that there is no definite report of the disease,
ed
although Liston showed its possible existence in the figure of his paper (Fig. 3-a). Here we reported seven cases of lower neck fistula. Features of lower neck fistula were as follows: 1) Most fistulas were situated on the left side since birth. 2) The site of
pt
the fistula orifice was at exactly the same point: near the sternoclavicular joint. The fistula orifice was situated at the anterior border of the sternocleidomastoid muscle and
Ac ce
on the line of the clavicular bone. 3) All of them had a short tract and a blind end in subcutaneous tissue. 4) Symptoms were seen in childhood and infection of the tract often occurred. 5) Their lining epithelium was mainly of ectodermal origin, consisting of a stratified squamous epithelium with skin adnexa. However, some fistulas contained ciliated columnar epithelium of endodermal origin. Considering these characteristics, we concluded that these fistulas should be viewed as one disease entity of congenital neck fistula. Four papers previously reported a very similar cutaneous fistula and all of them were published in dermatology journal(8-11). Those reports concluded that the fistulas were derived from ectodermal origin and they were designated as a dermoid fistula. We conjecture that the authors who insisted that the fistula was a dermoid fistula may not
Page 6 of 14
have kept the existence of the fourth branchial cleft remnant in mind because they were all dermatologists and may not have been familiar with branchial anomalies. The clinical characteristics of our case series were similar to those of the previously-reported cases(8-11). However, our cases differed from a dermoid fistula with regard to the
ip t
following points: 1) A dermoid fistula is usually situated on the midline of the body, and our case and the previously-reported cases were not situated on the midline. 2) The skin orifice of the fistulas was at the sternoclavicular joint which was near the anterior
cr
border of the sternocleidomastoid muscle. The second branchial remnant fistula was situated on the same border but usually at a more cranial side. Liston clearly stated
us
that the fourth branchial cleft remnant fistula theoretically opens on the anterior border of the sternocleidomastoid muscle. The textbook of human development by
an
Moore and Persaud(6) also suggested a possible fistula at the sternoclavicular joint (Fig. 3-b). 3) Among all reported cases(8-11) as well as our cases, the lesions were predominantly situated on the left side, which strongly suggested that the fistula was
M
related to the fourth branchial remnant fistula. 3) The lined epithelium in our cases 1 and 7 included a ciliated columnar epithelium (the epithelium was derived from
ed
endodermal origin). In case 1, a salivary gland was detected around the fistula. A dermoid fistula does not have these characteristics, and the branchial remnant fistula often has endodermal epithelium and a salivary gland is derived from the branchial
pt
arch. These points suggest that the cutaneous fistula reported herein is a fourth branchial cleft remnant and do not support that the fistula is a dermoid fistula. Of
Ac ce
course we cannot completely deny the possibility that these fistula is the second branchial cleft remnant but from its laterality and site we insist these fistula is more likely to be a fourth branchial cleft remnant.
Page 7 of 14
Conclusion We conclude that the seven cases of congenital lower neck fistula presented in this manuscript represent one disease entity, and judging from several clinical
ip t
characteristics, it may more likely be a fourth branchial cleft remnant fistula than a second branchial cleft remnant. Several similar cases had been reported in the past, but those authors considered the fistula to be a dermoid fistula. The present report is the
Ac ce
pt
ed
M
an
us
cr
first to insist that the fistula is a fourth branchial cleft remnant fistula.
Page 8 of 14
References 1.
Cote DN, Gianoli GJ. Fourth branchial cleft cysts. Otolaryngol Head Neck Surg 1996; 114: 95-97 Choi SS, Zalzal GH. Branchial anomalies: a review of 52 cases. Laryngoscope 1995; 105: 909-913
3.
Godin MS, Kearns DB, Pransky SM, et al. Fourth Branchial pouch sinus: principles
cr
of diagnosis and management. Laryngoscope 1990; 100: 174-178 4.
ip t
2.
Verret DJ, McClay J, Murray A, et al. Endoscopic cauterization of fourth branchial
5.
us
cleft sinus tract. Arch Otolaryngol Head Neck Surg 2004; 130: 465-468
Nicoucar K, Giger R, Pope Jr HG, et al. Management of congenital fourth branchial
an
arch anomalies: a review and analysis of published cases. J Pediatr Surg 2009; 44: 1432-1439
Moore KL and Persaud TVN. The pharyngeal organs. The Developing Human, 7th ed., Saunders, Philadelphia, 2007.
M
6.
Liston SL. Fourth branchial fistula. Otolaryngol Head Neck Surg 1981; 89: 520-522
8.
Matsunaga W, Ishihara T, Yasuno K. Congenital dermoid fistula of the anterior
ed
7.
chest region. Nishinihon J Dermatol 1994; 56: 34-39 9.
Muto J, Mori N, Konohara I, et al. Congenital dermoid fistula of the anterior chest
pt
region. Clin Exp Dermatol 2004; 29: 96-97 10. Numajiri T, Nishino K, Uenaka M, et al. Congenital dermoid fistula of the anterior
Ac ce
chest region. Acta Derm Venereol 2008; 88:538-539
11. Miyamoto T, Hosoda Y, Fujimoto Y, et al. Congenital skin fistula with sternal cleft. Br J Dermatol 1995; 132: 492-494
Page 9 of 14
Figure legends Figure 1. (a) The skin orifice of the fistula is situated near the sternoclavicular joint and
ip t
subcutaneous inflammation is seen around the fistula in this case. (case 1) (b) Preoperative contrast medium study shows that the fistula (arrow) runs toward the head of the left clavicular bone (arrowhead). (case 6)
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(c) Operative findings. The fistula is dilated like a cyst and then attenuates in the
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subcutaneous region. Finally, the fistula ends blindly there. (case 6)
Figure 2. Histopathological examination of the fistulas (a, 100X; b, 100X; c, 100X; d,
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40X), HE staining
(a) In most cases, the fistula was lined by stratified squamous epithelium. (case 1) (b) In case 7 the epithelium was mainly composed of ciliated columnar epithelium. The
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internal lumen contained a mucinous fluid.
(c) In case 1 the inner layer of the fistula changed to ciliated columnar epithelium in the
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deeper area.
in case 1.
Figure 3.
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(d) A salivary gland was detected around the ciliated columnar epithelium of the fistula
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(a) The theoretical entire course of the fourth branchial fistula. The figure also suggests the existence of a skin-side fistula (a figure cited from reference 7). The arrow shows a skin-sided fistula of the fourth branchial cleft. (b) Some possible opening sites of the branchial fistula or sinus are shown in the textbook of Moore and Persaud6). The arrow shows an external opening site that might have originated from the fourth branchial fistula near the sternoclavicular joint (a figure cited from reference 6).
Page 10 of 14
Table(s)
Table 1 Profile and characters of the seven patients Location
Length of fistula (mm)
Operation
Pathological findings
Stratified squamous epithelium, Fistulectomy ciliated columnar epitheium, salivary gland
－
Left
Female At birth
9y3m
－
Right
Sternoclavicular 6 joint
Fistulectomy
Stratified squamous epithelium, epidermal appendage
Male
2y7m
＋
Left
Sternoclavicular 11 joint
Fistulectomy
Stratified squamous epithelium, epidermal appendage
Left
Sternoclavicular 5 joint
Stratified squamous epithelium, Fistulectomy epidermal appendage, keratinous material
Left
Sternoclavicular 22 joint
Stratified squamous epithelium, Fistulectomy epidermal appendage, keratinous material
Left
Sternoclavicular 17 joint
Fistulectomy
Left
Sternoclavicular 15 joint
Fistulectomy Ciliated columnar epithelium
Female At birth
7m
＋
Male
At birth
6m
－
6
Male
At birth
1y5m
＋
7
Male
At birth
1y4m
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pt
5
＋
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At birth
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3
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2
At birth
ed
Male
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7m
Sternoclavicular 16 joint
1
4
Affected Side
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Case Gender Noticed age Treated age Infection
Stratified squamous epithelium, keratinous material
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Figure1
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Figure 1
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(b)
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Figure 2
(b)
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Figure(s)
(c)
(d)
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Figure 3
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Figure 3
(a)
(b)
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