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Cervical chondrocutaneous branchial remnants – Report of 17 cases
International Journal of Pediatric Otorhinolaryngology 78 (2014) 1961–1964
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Cervical chondrocutaneous branchial remnants – Report of 17 cases Ninoslav Begovic a, Radoje Simic a, Aleksandar Vlahovic a,*, Djordje Kravljanac a, Slavisa Djuricic b, Tanja Mijovic a a b
Division of Pediatric Surgery, Institute for Mother and Child Health Care of Serbia, Radoja Dakica 6-8, 11070 New Belgrade, Serbia Department of Clinical Pathology, Institute for Mother and Child Health Care of Serbia, Radoja Dakica 6-8, 11070 New Belgrade, Serbia
A R T I C L E I N F O
A B S T R A C T
Article history: Received 14 July 2014 Received in revised form 24 August 2014 Accepted 25 August 2014 Available online 1 September 2014
Background/purpose: Cervical chondrocutaneous branchial remnants are congenital, benign and rare neck masses. These anomalies are limited in the literature, reported mostly as case reports. Cervical chondrocutaneous branchial remnant is always present at birth, and the lesion is usually unilateral. Understanding and treatment of cervical chondrocutaneous branchial remnants requires knowledge of the related embryology. Methods: From January 2005 to December 2008, 17 patients with mean age of 32 months (range from 2 months to 15 years) with CCBRs were treated at the Division of Pediatric Surgery, Department of Plastic and Reconstructive Surgery and Burns at the Institute for Mother and Child Health Care, Belgrade, Serbia. The following objections were recorded: sex, lesion side, surgical data, associated malformations and pathohistology findings. Results: There were 7 females and 10 males, 4 with bilateral presences. Five children had associated anomalies, as follows: vesicoureteral reflux, atrial and ventricular septal defect, ventricular septal defect, branchiootorenal syndrome and preauricular sinus. There was a positive family history in one patient. Fifteen patients (88%) were treated with complete surgical excision and no connections with deep underlying structures of the neck were found. There were no complications at surgery. No recurrence was found during follow-up. Histopathology analysis revealed both, hyaline and elastic cartilage. Conclusion: Cervical chondrocutaneous branchial remnants are rare anomalies arising from branchial arch, probably originally from remnants of first or second arch. Surgical excision is the treatment of choice. From our experience, we suggest surgical treatment early in childhood because of esthetic reason, simplicity of the intervention and low complication rate. Also, the abdominal ultrasound and cardiac examination is recommended because of associated anomalies. ß 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Cervical Branchial Remnant Tragus Skin tag
1. Background/purpose Cervical chondrocutaneous branchial remnants (CCBRs) are congenital, benign and rare neck masses [1]. First description of CCBR was published in 1858 [2]. They are dysgenetic tumors, choristomas, originating from dislocated tissue. There are numerous synonyms for CCBRs such as ‘‘accessory tragus’’, ‘‘cervical skin tag’’, ‘‘wattle’’ or ‘‘cervical auricle’’ [3–7].
* Corresponding author. Tel.: +381 604112615. E-mail addresses: [email protected] (N. Begovic), [email protected] (R. Simic), [email protected] (A. Vlahovic), [email protected] (D. Kravljanac), [email protected] (S. Djuricic), [email protected] (T. Mijovic). http://dx.doi.org/10.1016/j.ijporl.2014.08.038 0165-5876/ß 2014 Elsevier Ireland Ltd. All rights reserved.
The only relatively large study (17 cases) was published in 1997 by Atlan et al. [8]. The term ‘‘cervical chondrocutaneous remnants’’ was suggested. The presentation of chondrocutaneous branchial remnants on the neck is uncommon, compared with preauricular tragus which is more common. Bilateral appearance is very rare [5–11]. The embryogenesis of these anomalies is controversial. There are several theories of embryogenesis of branchial anomalies, but the most accepted theory proposes that they are vestigial remnants, resulting from incomplete obliteration of the branchial apparatus or buried epithelial cell rests. Some authors suggested that they arise from ectopic auricular tissue [1,8], while others are convinced that they originate from branchial tissue contributing to the formation of most cervical structures [4,9]. The presence of elastic or hyaline cartilage may suggest the origin of CCBR. The
N. Begovic et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1961–1964
1962
presence of elastic cartilage may suggest an auricular origin (first or second branchial arch). On the other hand, the finding of hyaline cartilage will exclude an auricular and favor a cervical origin (from second or lower branchial arches) [12,13]. 2. Materials and methods The study was carried out on the Division of Pediatric Surgery, Department of Plastic and Reconstructive Surgery and Burns at the Institute for Mother and Child Health Care of Serbia in Belgrade, from January 2005 to December 2008. Diagnosis of CCBR was made on the basis of clinical presentation and pathohistological findings of operated patients. The clinical records and histology reports of all patients were retrospectively reviewed. We analyzed sex, lesion side, surgical data, associated malformations and histopathology findings.
Fig. 1. A photo showing a bilateral CCBR in 7 year-old boy.
3. Results Seventeen consecutive patients (10 male) with mean age of 32 months (range from 2 months to 15 years) were treated because of CCBRs (Table 1). Tags were located in the lower half of the neck, over or near the sternocleidomastoid muscle, eight on the left and five on the right side. There were four patients with bilateral appearance (Fig. 1). Before the intervention, the abdominal ultrasound examination was performed in all the patients. In five patients (29.5%) associated anomalies were found: vesico-ureteral reflux (VUR) in a 15-month-old female; branchio-oto-renal syndrome (BOR) in a 9-month-old male (Fig. 2); and two cardiac anomalies (female infant had atrial septal defect (ASD) and ventricular septal defect (VSD) and 7-year-old boy with corrected VSD). A female infant with ASD and VSD died before surgery. One female with bilateral lesions had associated preauricular sinus on the left side which was excised. The oldest patient had ingrown nail surgery simultaneously. Surgical intervention was performed in 15 children. The mean age was 32 months (range from 2 months to 15 years). In all cases simple excision of CCBR was performed in general anesthesia. The masses and underlying cartilage were excised with a part of subcutaneous tissue. The cartilage did not reach deeper than the surface of the neck musculature and no connections with deep underlying structures of the neck were found. All excised masses were pedunculated, skin-colored with central presence of
Fig. 2. Right sided cervical chondrocutaneous branchial remnant associated with microtia in BOR syndrome.
cartilage core (Fig. 3). All lesions were cured with a single operation (follow-up lasted from 3 month to 48 months, median 13 months). Pathologic examination confirmed the presence of two different cartilage types – hyaline cartilage in eight (53%) and elastic in seven (47%) patients. No recurrences were found during the follow-up.
Table 1 Summary of patient data. Patient
Age at surgery (months)
Sex
Side
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17
2 5 6 6 7 7 7 8 9 13 15 54 (4.5 years) 84 (7 years) 84 (7 years) 180 (15 years) Not operated (withdrawn consent) Not operated (died prior surgical treatment)
Male Female Female Male Female Female Female Male Male Male Male Female Male Male Male Male Female
Left Left Right Left Left Left Bilateral Bilateral Right Left Right Right Left Bilateral Right Bilateral Left
a b c d
Branchio-oto-renal syndrome. Vesicoureteral reflux. Ventricular septal defect. Atrial septal defect.
Associated anomalies
Sinus preauricularis Sy. BORa VURb
VSDc
ASDd, VSD
Histology Hyaline Hyaline Hyaline Elastic Elastic Elastic Elastic Elastic Elastic Hyaline Hyaline Hyaline Hyaline Elastic Hyaline
N. Begovic et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1961–1964
Fig. 3. Intraoperative photo of cervical chondrocutaneous branchial remnant showing the cartilage core.
4. Discussion Most of the congenital branchial anomalies found in the cervical area are cysts, sinuses or fistulas. CCBRs are rare in contrast to the similar preauricular tags [3]. The name cervical chondrocutaneous branchial remnant was proposed by Atlan et al. [8] in order to mark it as a single entity, contrary to many different terms used before. The term CCBR is now widely accepted and it eliminates diagnostic confusion in the most cases. CCBRs presented 32% of all branchial anomalies that were operated at the our Division during the study period. This is a large percentage, having in mind quite rare appearance of this anomaly. We excised 18 tags in 15 children, 10 on the left, and eight on the right side of the neck. In remaining two children the CCBR was diagnosed, however, without surgical treatment because a female infant with left sided CCBR died due to respiratory distress syndrome prior to surgical intervention. The last case (male with bilateral tags) parents withdrew consent for the operation. According to the available literature, we are reporting one of the largest series of CCBRs [1–22]. In 1997, Atlan et al. [8] reported case study of CCBRs and described their predominance in males, scarcity of bilateral lesions and a high incidence of associated anomalies. Similarly, in our series there was also male predominance (58.8%), as well as left side predominance (46.7%). In the edited English literature incidence of bilateral appearances is rare. Until now there are 9 reported cases [3,7–9,11,14–16]. In our study we had four children with bilateral presentation of CCBRs. This is a relatively high percentage comparing to other authors [3,7–9,11,14–16]. Also, one thing is interestingly: we found a one case of inheritance (father and son) and that, by our knowledge, was not reported previously in the literature. In our study 5 of 17 children had associated anomalies. In the study of patients described by Atlan et al. [8] there was a high incidence of associated anomalies (13 of 17 children). However, in other papers, mainly case report, there were no associated anomalies [1,3,7,9–11,15,17–19]. One child in our study had VUR. In the study by Atlan et al. [8] there were 2 patients with genitourinary tract abnormality (ultrasound was performed in 6 children). Although the incidence of associated urinary tract malformations is not very high, we agree with Atlan et al. that abdominal ultrasound should be performed in every child with CCBR. This diagnostic procedure can help in detecting urinary tract anomalies before they are manifested with urinary tract infections, and consequent renal damage. One child had associated BOR syndrome (an autosomal dominant disorder involving branchial arch anomalies linked to
1963
a defect in chromosome 8q) with preauricular sinus on the left side, lateral neck fistula on the left side and structural defect of the external ear on the right side, without renal malformations [20]. Several authors reported different associated ear malformations: microtia and stenosis of the external auditory canal [4]; low position of the ear [21]. Chander et al. [22] described on autopsy of a 30-week-old male fetus with hydrocephalus, left microtia, bilateral lip and palate cleft, bilateral nasal ala agenesis, thymic hypoplasia and dextrocardia. We had two children with cardiac anomalies – one child with ASD and VSD, and another with VSD. Cardiac examinations in these children were performed independently from our study because of clinical signs related to the anomalies. Association between CCBRs and cardiac anomalies was also reported by Rameh et al. [16]. Based on this, cardiac examination is recommended in all children with CCBRs. Tamir et al. [10] suggested operation just before starting school which allows minimization of surgical complications and spares the child of the psychological complications. Similar, general practice in our hospital is to perform intervention in the early childhood due to several reasons: (1) esthetic; (2) to elude psychological complications; (3) social component and (4) histopathological verification of the lesions. Also, pediatric anesthesiologists role is crucial in making assessment when the child is fit for general anesthesia. According to Atlan at al. in all 15 operated patients elastic cartilage was found. In contrast to this finding, we had 7 children with elastic and 8 with hyaline cartilages. In various case reports both types of cartilage were found – elastic in four cases [1,3,4,19] and hyaline in two [10,21]. Other authors did not notify the type of cartilage they have found. The finding of hyaline cartilage would exclude an auricular origin and favor a cervical origin from the second or lower branchial arch. The finding of both cartilage types supports the theory that CCBRs derive from the first and the second branchial arch [3,10]. 5. Conclusion Cervical chondrocutaneous branchial remnants are rare anomalies arising from branchial arch, probably from remnants of the first or second arch. They do not communicate with other structures of the neck; always have central cartilage, elastic or hyaline. The treatment of choice is complete surgical excision. We propose operation early in the childhood (from 2 to 6 years), because of esthetic and social reasons. Also, the abdominal ultrasound and cardiac examination is recommended because of associated anomalies. These lesions are benign, which is very important information for the patients with CCBRs, and their parents. References [1] T.A. van Kalkeren, A. Frima van Aerem, E.J.M. Ahsmann, H.F. Mahieu, Case report of a unilateral cervical chondrocutaneous branchial remnant, Int. J. Pediatr. Otorhynolaryngol. Extra 2 (2007) 225–227. [2] J. Birkett, Congenital supernumerary and imperfectly developed auricles on the sides of the neck, Trans. Pathol. Soc. Lond. 9 (1858) 448. [3] T. Ozturk, T. Ozdemir, S. Demirbag, C. Atabek, I. Surer, M. Safali, et al., Bilateral cervical chondrocutaneous remnants: a case report and review of the literature, Turk. J. Pediatr. 48 (2006) 175–177. [4] E. Bendet, A wattle (cervical accesory tragus), Otolaryngol. Head Neck Surg. 121 (1999) 508–509. [5] M.U. Akyol, Accessory auricle, Otolaryngol. Head Neck Surg. 122 (2000) 155. [6] C.R. Rund, S.W. Galyon, E.G. Fischer, Pathologic quiz case: an anterior neck mass in a 5-month-old female infant. Wattle (congenital cervical tragus), Arch. Pathol. Lab. Med. 128 (2004) 1453–1454. [7] D. Dayal, P. Menon, Bilateral cervical chondrocutaneous remnants, Indian Pediatr. 45 (2008) 221. [8] G. Atlan, E.P. Egerszegi, P. Brochu, L. Caouette-Laberge, P. Bortoluzzi, Cervical chondrocutaneous branchial remnants, Plast. Reconstr. Surg. 100 (1997) 32–39.
1964
N. Begovic et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1961–1964
[9] H. Braun, T. Hofmann, H. Wolfgruber, W. Anderhuber, A. Beham, H. Stammberger, Case report of bilateral chonrocutaneous branchial remnants, Int. J. Pediatr. Otorhynolaryngol. 67 (2003) 89–92. [10] S. Tamir, M. Nidal, R. Constantin, R. Perez, J.-Y. Sichel, Bilateral cervical chondrocutaneous remnants, Int. J. Pediatr. Otorhinolaryngol. Extra 3 (2008) 117–119. [11] H.A. Nasser, F. Iskandrani, T. Berjaoni, S. Fleifel, A case report of bilateral cervical chondrocutaneous remnants with review of the literature, J. Pediatr. Surg. 46 (2011) 998–1000. [12] T.M. Benson, K. Dalen, A.A. Mancuso, H.H. Kerr, A.A. Cacciarelli, M.F. Mafee, Congenital anomalies of the branchial apparatus: embryology and pathologic anatomy, Radiographics 12 (1992) 943–960. [13] W.J. Schroeder, N. Mohyuddin, J. Maddalozzo, Branchial anomalies in the pediatric population, Otolaryngol. Head Neck Surg. 137 (2007) 289–295. [14] B. Coras, C. Hafner, A. Roesch, T. Vogt, M. Landthaler, U. Hohenleutner, Congenital cartilaginous rest of the neck (wattles), Dermatol. Surg. 31 (2005) 1349–1350. [15] Y. Gilboa, R. Achiron, Y. Zalel, M. Bronshtein, Prenatal diagnosis of cervical chondrocutaneous vestige, Ultrasound Obstet. Gynecol. 30 (2007) 1010–1012.
[16] S. Marti, Cervical auricles from first and second branchial arc, Indian J. Surg. 68 (2006) 35–37. [17] S. Rai, C. Monohar, Pathologic quiz case: subcutaneous nodule in the neck. Congenital cartilaginous rest, Arch. Pathol. Lab. Med. 127 (2003) 438–439. [18] B. Hemmaoui, N. Fejjal, K. Nadour, F. Benariba, Cervical chondrocutaneous branchial remnant. Two cases report, Indian J. Paediatr. Dermatol. 14 (2013) 33–35. [19] C. Rameh, C. Sidani, M. Arabi, J. Achkar, Bilateral cervical chondrocutaneous branchial remnants associated with cardiac anomalies, J. Otolaryngol. 36 (2007) 79–81 (Da li imacitieran u textu). [20] E.K. Anderson, E.J.B. Hartley, M.C. Myer, An asymptomatic infant with neck mass, Clin. Pediatr. 40 (2001) 41–43. [21] B. Fuad, C. Elmir, D.D. Samir, Neck auricles with microtia and low position of the right ear: a case report, Auris Nasus Larynx 30 (2003) 283–285. [22] B. Chander, S.S. Dogra, R. Raina, C. Sharma, R. Sharma, Chondrocutaneous branchial remnants or cartilaginous choristoma: terminology, biological behaviour and salience of bilateral cervical lesions, Turk. Patoloji Derg. 4 (2014) 1–6.
Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Cervical chondrocutaneous branchial remnants – Report of 17 cases Ninoslav Begovic a, Radoje Simic a, Aleksandar Vlahovic a,*, Djordje Kravljanac a, Slavisa Djuricic b, Tanja Mijovic a a b
Division of Pediatric Surgery, Institute for Mother and Child Health Care of Serbia, Radoja Dakica 6-8, 11070 New Belgrade, Serbia Department of Clinical Pathology, Institute for Mother and Child Health Care of Serbia, Radoja Dakica 6-8, 11070 New Belgrade, Serbia
A R T I C L E I N F O
A B S T R A C T
Article history: Received 14 July 2014 Received in revised form 24 August 2014 Accepted 25 August 2014 Available online 1 September 2014
Background/purpose: Cervical chondrocutaneous branchial remnants are congenital, benign and rare neck masses. These anomalies are limited in the literature, reported mostly as case reports. Cervical chondrocutaneous branchial remnant is always present at birth, and the lesion is usually unilateral. Understanding and treatment of cervical chondrocutaneous branchial remnants requires knowledge of the related embryology. Methods: From January 2005 to December 2008, 17 patients with mean age of 32 months (range from 2 months to 15 years) with CCBRs were treated at the Division of Pediatric Surgery, Department of Plastic and Reconstructive Surgery and Burns at the Institute for Mother and Child Health Care, Belgrade, Serbia. The following objections were recorded: sex, lesion side, surgical data, associated malformations and pathohistology findings. Results: There were 7 females and 10 males, 4 with bilateral presences. Five children had associated anomalies, as follows: vesicoureteral reflux, atrial and ventricular septal defect, ventricular septal defect, branchiootorenal syndrome and preauricular sinus. There was a positive family history in one patient. Fifteen patients (88%) were treated with complete surgical excision and no connections with deep underlying structures of the neck were found. There were no complications at surgery. No recurrence was found during follow-up. Histopathology analysis revealed both, hyaline and elastic cartilage. Conclusion: Cervical chondrocutaneous branchial remnants are rare anomalies arising from branchial arch, probably originally from remnants of first or second arch. Surgical excision is the treatment of choice. From our experience, we suggest surgical treatment early in childhood because of esthetic reason, simplicity of the intervention and low complication rate. Also, the abdominal ultrasound and cardiac examination is recommended because of associated anomalies. ß 2014 Elsevier Ireland Ltd. All rights reserved.
Keywords: Cervical Branchial Remnant Tragus Skin tag
1. Background/purpose Cervical chondrocutaneous branchial remnants (CCBRs) are congenital, benign and rare neck masses [1]. First description of CCBR was published in 1858 [2]. They are dysgenetic tumors, choristomas, originating from dislocated tissue. There are numerous synonyms for CCBRs such as ‘‘accessory tragus’’, ‘‘cervical skin tag’’, ‘‘wattle’’ or ‘‘cervical auricle’’ [3–7].
* Corresponding author. Tel.: +381 604112615. E-mail addresses: [email protected] (N. Begovic), [email protected] (R. Simic), [email protected] (A. Vlahovic), [email protected] (D. Kravljanac), [email protected] (S. Djuricic), [email protected] (T. Mijovic). http://dx.doi.org/10.1016/j.ijporl.2014.08.038 0165-5876/ß 2014 Elsevier Ireland Ltd. All rights reserved.
The only relatively large study (17 cases) was published in 1997 by Atlan et al. [8]. The term ‘‘cervical chondrocutaneous remnants’’ was suggested. The presentation of chondrocutaneous branchial remnants on the neck is uncommon, compared with preauricular tragus which is more common. Bilateral appearance is very rare [5–11]. The embryogenesis of these anomalies is controversial. There are several theories of embryogenesis of branchial anomalies, but the most accepted theory proposes that they are vestigial remnants, resulting from incomplete obliteration of the branchial apparatus or buried epithelial cell rests. Some authors suggested that they arise from ectopic auricular tissue [1,8], while others are convinced that they originate from branchial tissue contributing to the formation of most cervical structures [4,9]. The presence of elastic or hyaline cartilage may suggest the origin of CCBR. The
N. Begovic et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1961–1964
1962
presence of elastic cartilage may suggest an auricular origin (first or second branchial arch). On the other hand, the finding of hyaline cartilage will exclude an auricular and favor a cervical origin (from second or lower branchial arches) [12,13]. 2. Materials and methods The study was carried out on the Division of Pediatric Surgery, Department of Plastic and Reconstructive Surgery and Burns at the Institute for Mother and Child Health Care of Serbia in Belgrade, from January 2005 to December 2008. Diagnosis of CCBR was made on the basis of clinical presentation and pathohistological findings of operated patients. The clinical records and histology reports of all patients were retrospectively reviewed. We analyzed sex, lesion side, surgical data, associated malformations and histopathology findings.
Fig. 1. A photo showing a bilateral CCBR in 7 year-old boy.
3. Results Seventeen consecutive patients (10 male) with mean age of 32 months (range from 2 months to 15 years) were treated because of CCBRs (Table 1). Tags were located in the lower half of the neck, over or near the sternocleidomastoid muscle, eight on the left and five on the right side. There were four patients with bilateral appearance (Fig. 1). Before the intervention, the abdominal ultrasound examination was performed in all the patients. In five patients (29.5%) associated anomalies were found: vesico-ureteral reflux (VUR) in a 15-month-old female; branchio-oto-renal syndrome (BOR) in a 9-month-old male (Fig. 2); and two cardiac anomalies (female infant had atrial septal defect (ASD) and ventricular septal defect (VSD) and 7-year-old boy with corrected VSD). A female infant with ASD and VSD died before surgery. One female with bilateral lesions had associated preauricular sinus on the left side which was excised. The oldest patient had ingrown nail surgery simultaneously. Surgical intervention was performed in 15 children. The mean age was 32 months (range from 2 months to 15 years). In all cases simple excision of CCBR was performed in general anesthesia. The masses and underlying cartilage were excised with a part of subcutaneous tissue. The cartilage did not reach deeper than the surface of the neck musculature and no connections with deep underlying structures of the neck were found. All excised masses were pedunculated, skin-colored with central presence of
Fig. 2. Right sided cervical chondrocutaneous branchial remnant associated with microtia in BOR syndrome.
cartilage core (Fig. 3). All lesions were cured with a single operation (follow-up lasted from 3 month to 48 months, median 13 months). Pathologic examination confirmed the presence of two different cartilage types – hyaline cartilage in eight (53%) and elastic in seven (47%) patients. No recurrences were found during the follow-up.
Table 1 Summary of patient data. Patient
Age at surgery (months)
Sex
Side
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17
2 5 6 6 7 7 7 8 9 13 15 54 (4.5 years) 84 (7 years) 84 (7 years) 180 (15 years) Not operated (withdrawn consent) Not operated (died prior surgical treatment)
Male Female Female Male Female Female Female Male Male Male Male Female Male Male Male Male Female
Left Left Right Left Left Left Bilateral Bilateral Right Left Right Right Left Bilateral Right Bilateral Left
a b c d
Branchio-oto-renal syndrome. Vesicoureteral reflux. Ventricular septal defect. Atrial septal defect.
Associated anomalies
Sinus preauricularis Sy. BORa VURb
VSDc
ASDd, VSD
Histology Hyaline Hyaline Hyaline Elastic Elastic Elastic Elastic Elastic Elastic Hyaline Hyaline Hyaline Hyaline Elastic Hyaline
N. Begovic et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1961–1964
Fig. 3. Intraoperative photo of cervical chondrocutaneous branchial remnant showing the cartilage core.
4. Discussion Most of the congenital branchial anomalies found in the cervical area are cysts, sinuses or fistulas. CCBRs are rare in contrast to the similar preauricular tags [3]. The name cervical chondrocutaneous branchial remnant was proposed by Atlan et al. [8] in order to mark it as a single entity, contrary to many different terms used before. The term CCBR is now widely accepted and it eliminates diagnostic confusion in the most cases. CCBRs presented 32% of all branchial anomalies that were operated at the our Division during the study period. This is a large percentage, having in mind quite rare appearance of this anomaly. We excised 18 tags in 15 children, 10 on the left, and eight on the right side of the neck. In remaining two children the CCBR was diagnosed, however, without surgical treatment because a female infant with left sided CCBR died due to respiratory distress syndrome prior to surgical intervention. The last case (male with bilateral tags) parents withdrew consent for the operation. According to the available literature, we are reporting one of the largest series of CCBRs [1–22]. In 1997, Atlan et al. [8] reported case study of CCBRs and described their predominance in males, scarcity of bilateral lesions and a high incidence of associated anomalies. Similarly, in our series there was also male predominance (58.8%), as well as left side predominance (46.7%). In the edited English literature incidence of bilateral appearances is rare. Until now there are 9 reported cases [3,7–9,11,14–16]. In our study we had four children with bilateral presentation of CCBRs. This is a relatively high percentage comparing to other authors [3,7–9,11,14–16]. Also, one thing is interestingly: we found a one case of inheritance (father and son) and that, by our knowledge, was not reported previously in the literature. In our study 5 of 17 children had associated anomalies. In the study of patients described by Atlan et al. [8] there was a high incidence of associated anomalies (13 of 17 children). However, in other papers, mainly case report, there were no associated anomalies [1,3,7,9–11,15,17–19]. One child in our study had VUR. In the study by Atlan et al. [8] there were 2 patients with genitourinary tract abnormality (ultrasound was performed in 6 children). Although the incidence of associated urinary tract malformations is not very high, we agree with Atlan et al. that abdominal ultrasound should be performed in every child with CCBR. This diagnostic procedure can help in detecting urinary tract anomalies before they are manifested with urinary tract infections, and consequent renal damage. One child had associated BOR syndrome (an autosomal dominant disorder involving branchial arch anomalies linked to
1963
a defect in chromosome 8q) with preauricular sinus on the left side, lateral neck fistula on the left side and structural defect of the external ear on the right side, without renal malformations [20]. Several authors reported different associated ear malformations: microtia and stenosis of the external auditory canal [4]; low position of the ear [21]. Chander et al. [22] described on autopsy of a 30-week-old male fetus with hydrocephalus, left microtia, bilateral lip and palate cleft, bilateral nasal ala agenesis, thymic hypoplasia and dextrocardia. We had two children with cardiac anomalies – one child with ASD and VSD, and another with VSD. Cardiac examinations in these children were performed independently from our study because of clinical signs related to the anomalies. Association between CCBRs and cardiac anomalies was also reported by Rameh et al. [16]. Based on this, cardiac examination is recommended in all children with CCBRs. Tamir et al. [10] suggested operation just before starting school which allows minimization of surgical complications and spares the child of the psychological complications. Similar, general practice in our hospital is to perform intervention in the early childhood due to several reasons: (1) esthetic; (2) to elude psychological complications; (3) social component and (4) histopathological verification of the lesions. Also, pediatric anesthesiologists role is crucial in making assessment when the child is fit for general anesthesia. According to Atlan at al. in all 15 operated patients elastic cartilage was found. In contrast to this finding, we had 7 children with elastic and 8 with hyaline cartilages. In various case reports both types of cartilage were found – elastic in four cases [1,3,4,19] and hyaline in two [10,21]. Other authors did not notify the type of cartilage they have found. The finding of hyaline cartilage would exclude an auricular origin and favor a cervical origin from the second or lower branchial arch. The finding of both cartilage types supports the theory that CCBRs derive from the first and the second branchial arch [3,10]. 5. Conclusion Cervical chondrocutaneous branchial remnants are rare anomalies arising from branchial arch, probably from remnants of the first or second arch. They do not communicate with other structures of the neck; always have central cartilage, elastic or hyaline. The treatment of choice is complete surgical excision. We propose operation early in the childhood (from 2 to 6 years), because of esthetic and social reasons. Also, the abdominal ultrasound and cardiac examination is recommended because of associated anomalies. These lesions are benign, which is very important information for the patients with CCBRs, and their parents. References [1] T.A. van Kalkeren, A. Frima van Aerem, E.J.M. Ahsmann, H.F. Mahieu, Case report of a unilateral cervical chondrocutaneous branchial remnant, Int. J. Pediatr. Otorhynolaryngol. Extra 2 (2007) 225–227. [2] J. Birkett, Congenital supernumerary and imperfectly developed auricles on the sides of the neck, Trans. Pathol. Soc. Lond. 9 (1858) 448. [3] T. Ozturk, T. Ozdemir, S. Demirbag, C. Atabek, I. Surer, M. Safali, et al., Bilateral cervical chondrocutaneous remnants: a case report and review of the literature, Turk. J. Pediatr. 48 (2006) 175–177. [4] E. Bendet, A wattle (cervical accesory tragus), Otolaryngol. Head Neck Surg. 121 (1999) 508–509. [5] M.U. Akyol, Accessory auricle, Otolaryngol. Head Neck Surg. 122 (2000) 155. [6] C.R. Rund, S.W. Galyon, E.G. Fischer, Pathologic quiz case: an anterior neck mass in a 5-month-old female infant. Wattle (congenital cervical tragus), Arch. Pathol. Lab. Med. 128 (2004) 1453–1454. [7] D. Dayal, P. Menon, Bilateral cervical chondrocutaneous remnants, Indian Pediatr. 45 (2008) 221. [8] G. Atlan, E.P. Egerszegi, P. Brochu, L. Caouette-Laberge, P. Bortoluzzi, Cervical chondrocutaneous branchial remnants, Plast. Reconstr. Surg. 100 (1997) 32–39.
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N. Begovic et al. / International Journal of Pediatric Otorhinolaryngology 78 (2014) 1961–1964
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