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A case report of bilateral cervical chondrocutaneous remnants with review of the literature
Journal of Pediatric Surgery (2011) 46, 998–1000
www.elsevier.com/locate/jpedsurg
A case report of bilateral cervical chondrocutaneous remnants with review of the literature Haydar A. Nasser a,⁎, Fadi Iskandarani a , Tarek Berjaoui b , Siham Fleifel c a
Pediatric Surgery Division, Rafic Hariri University Hospital, Beirut, Lebanon Department of General Surgery, Rafic Hariri University Hospital, Beirut, Lebanon c Histopathology Division, Rafic Hariri University Hospital, Beirut, Lebanon b
Received 30 October 2010; revised 12 January 2011; accepted 16 January 2011
Key words: Chondrocutaneous Remnants; Cervical; Bilateral; Wattle; Tragus
Abstract Cervical chondrocutaneous remnants are very rare entities. They are thought to originate either from the second branchial arch or from auricular tissues. To date, less than 40 cases have been reported in the medical literature, and only 7 cases were bilateral. We report the case of a 1-month-old girl presenting with bilateral neck lesions since birth, with no other anomalies. Complete surgical excision was performed, the pathology of which confirmed the diagnosis of chondrocutaneous remnants. Followup after 9 months showed no clinical evidence of complications or recurrence. © 2011 Elsevier Inc. All rights reserved.
Congenital neoplasms of the head and neck are usually teratomas, with cysts and sinuses being the most frequent anomalies [1]. Cervical chondrocutaneous remnants are uncommon congenital lesions of the lateral neck and appear similar to “accessory tragi.” Bilateral lesions are very rare [2]. Only 7 cases of bilateral cervical chondrocutaneous remnants were reported in the English medical literature [2-7].
1. Case report A 1-month-old girl was referred to pediatric surgery clinics because of bilateral neck lesions noticed since birth. Both lesions were situated at the anterolateral aspect of the lower neck (Fig. 1) and were covered by normal skin, each ⁎ Corresponding author. Tel.: +961 3 972884. E-mail address: [email protected] (H.A. Nasser). 0022-3468/$ – see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2011.01.015
measuring about 2 cm in diameter, elastic, and mobile in relation to its underlying structures. There were no overlying epidermal pits, nor were there any inflammatory changes. Both lesions were misdiagnosed as branchial cleft cysts after clinical evaluation in an outside hospital. The rest of the findings from the physical examination was normal because there was no evidence of any dysmorphic or urogenital anomalies. Cardiac auscultation revealed normal heart sounds with no evidence of any pathologic murmurs. During surgical excision under general anesthesia, it was noticed that both the right and left lesions were firmly adherent to the fascia of the sternocleidomastoid muscle. The right lesion measured 2.5 × 0.8 × 0.3 cm (Fig. 2), whereas the left one measured 2 × 0.5 × 0.3 cm. Histologically, the lesions consisted of adipose tissue, hair follicles, and exocrine glands, covered by normal skin. Hematoxylin and eosin staining of the tissue demonstrated central islands of chondrocytes within the deep dermis (Fig. 3). The differential diagnosis of such cartilaginous tissue within a congenital lesion could include a type 2 first
Case report of bilateral cervical chondrocutaneous remnants
Fig. 1 A photo showing the bilateral cervical chondrocutaneous remnants.
branchial cleft anomaly or chondrocutaneous remnants within the skin (choristoma). A follow-up visit done 9 months postoperatively revealed the patient to be in good health, with no evidence of complications or recurrence of the lesions.
2. Discussion The most common diagnosis of congenital cystic lesions presenting within the neck is that of the second branchial arch anomalies [8]. Chondrocutaneous remnants, which are also thought to arise from the second arch, are very rare. We searched the English medical literature using the Medline and Pubmed databases from January 1966 through December 2010. Only 7 cases of bilateral cervical chondrocutaneous remnants have been reported [2-7]. In 1988, 7 cases of “cervical skin tags” were reported [7], 2 of which were bilateral branchial remnants. The largest case series of chondrocutaneous remnants was reported by Atlan et al in 1997 [6]. They reviewed 20 cases of cervical tags, of which 17 were true cervical chondrocutaneous branchial remnants. They established an association of these choristomas with other malformations, which was in contrast to the previously published case reports. Only 1 case was bilateral in nature. The most common associated malformations were oral (cleft palate and oronasal reflux), auditory (neurosensory deafness, serous otitis media, and external ear
999
Fig. 3 Pathology image showing the cartilage band (C) beneath the skin epidermis (E) and adipose tissue (A). Hair follicles are also evident (H).
malformations), respiratory (tracheomalacia), gastrointestinal (inguinal hernia), genitourinary (hydronephrosis), and cardiovascular (atrial septal defect) [6]. The fact that these lesions were classified under different names (tragi, wattles, rests, choristomas, vestiges, etc) reflects the poor comprehension of their pathology. Because of their resemblance to accessory tragus, some authors have postulated that the origin of these lesions is the pinna rests of the ear [6,9], whereas others suggested a branchial arch origin [10]. According to Atlan et al, both theories are plausible; however, he preferred to divide chondrocutaneous remnants into 2 subtypes according to their location and the type of cartilage they contain [6].The presence of an elastic cartilage directs one to believe that these lesions are auricular in origin, whereas their lower location below the hyoid bone and their association with hyaline cartilage suggests a second branchial arch origin. The clinical characteristics of cervical chondrocutaneous branchial remnants are as follows: male predominance, presence at birth, and location in the middle or lower third of the neck anterior to the sternocleidomastoid muscle. Their skin is similar to the neck skin. The lesions are painless with no erythema or discharge [2]. The reported high incidence of associated anomalies mandates a meticulous physical examination of patients with chondrocutaneous remnants [4,6]. Cervical and preauricular remnants were in some instances associated with Goldenhar, Treacher-Collins, Nager, and some other well-characterized syndromes [3]. Our case was not associated with other congenital anomalies. The treatment of choice adopted in our case and in all published cases was local excision, with an excellent postoperative outcome.
3. Conclusion Fig. 2
Excised left chondrocutaneous remnant.
Cervical chondrocutaneous remnants are rare entities, and the occurrence of a bilateral case is even rarer. Only 7
1000 bilateral cases were reported in the medical literature, and the current one is to be added. Pediatricians, pediatric surgeons, and pathologists should be aware of this entity because of the risk of associated anomalies that can be detected by a detailed physical examination. Local excision seems to be the best treatment modality.
References [1] Park YW. Evaluation of neck masses in children. Am Fam Physician 1995;51:1904-12. [2] Oztürk H, Ozdemir T, Demirbağ S, et al. Bilateral cervical chondrocutaneous remnants: a case report and review of the literature. Turk J Pediatr 2006;48:175-7.
H.A. Nasser et al. [3] Dayal D, Menon P. Bilateral cervical chondrocutaneous branchial remnants. Indian Pediatr 2008;45:221. [4] Rameh C, Sidani C, Arabi M, et al. Bilateral cervical chondrocutaneous branchial remnants associated with cardiac anomalies. J Otolaryngol 2007;36:E79-E81. [5] Braun H, Hofmann T, Wolfgruber H, et al. Case report of bilateral cervical chondrocutaneous branchial remnants. Int J Pediatr Otorhinolaryngol 2003;67:89-92. [6] Atlan G, Egerszegi EP, Brochu P, et al. Cervical chondrocutaneous branchial remnants. Plast Reconstr Surg 1997;100:32-9. [7] Doi O, Hutson JM, Myers NA, et al. Branchial remnants: a review of 58 cases. J Pediatr Surg 1988;23:789-92. [8] Acierno SP, Waldhausen JH: Congenital cervical cysts, sinuses and fistulae. Otolaryngol Clin North Am 2007;40:161-76, vii-viii. [9] Champion RH, Burton JL, Burns DA. Rook/Wilkinson/Ebling. Textbook of dermatology. 5th ed. Oxford: Blackwell-Scientific; 1994. [10] Bendet E. A wattle (cervical accessory tragus). Otolaryngol Head Neck Surg 1999;121:508-9.
www.elsevier.com/locate/jpedsurg
A case report of bilateral cervical chondrocutaneous remnants with review of the literature Haydar A. Nasser a,⁎, Fadi Iskandarani a , Tarek Berjaoui b , Siham Fleifel c a
Pediatric Surgery Division, Rafic Hariri University Hospital, Beirut, Lebanon Department of General Surgery, Rafic Hariri University Hospital, Beirut, Lebanon c Histopathology Division, Rafic Hariri University Hospital, Beirut, Lebanon b
Received 30 October 2010; revised 12 January 2011; accepted 16 January 2011
Key words: Chondrocutaneous Remnants; Cervical; Bilateral; Wattle; Tragus
Abstract Cervical chondrocutaneous remnants are very rare entities. They are thought to originate either from the second branchial arch or from auricular tissues. To date, less than 40 cases have been reported in the medical literature, and only 7 cases were bilateral. We report the case of a 1-month-old girl presenting with bilateral neck lesions since birth, with no other anomalies. Complete surgical excision was performed, the pathology of which confirmed the diagnosis of chondrocutaneous remnants. Followup after 9 months showed no clinical evidence of complications or recurrence. © 2011 Elsevier Inc. All rights reserved.
Congenital neoplasms of the head and neck are usually teratomas, with cysts and sinuses being the most frequent anomalies [1]. Cervical chondrocutaneous remnants are uncommon congenital lesions of the lateral neck and appear similar to “accessory tragi.” Bilateral lesions are very rare [2]. Only 7 cases of bilateral cervical chondrocutaneous remnants were reported in the English medical literature [2-7].
1. Case report A 1-month-old girl was referred to pediatric surgery clinics because of bilateral neck lesions noticed since birth. Both lesions were situated at the anterolateral aspect of the lower neck (Fig. 1) and were covered by normal skin, each ⁎ Corresponding author. Tel.: +961 3 972884. E-mail address: [email protected] (H.A. Nasser). 0022-3468/$ – see front matter © 2011 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2011.01.015
measuring about 2 cm in diameter, elastic, and mobile in relation to its underlying structures. There were no overlying epidermal pits, nor were there any inflammatory changes. Both lesions were misdiagnosed as branchial cleft cysts after clinical evaluation in an outside hospital. The rest of the findings from the physical examination was normal because there was no evidence of any dysmorphic or urogenital anomalies. Cardiac auscultation revealed normal heart sounds with no evidence of any pathologic murmurs. During surgical excision under general anesthesia, it was noticed that both the right and left lesions were firmly adherent to the fascia of the sternocleidomastoid muscle. The right lesion measured 2.5 × 0.8 × 0.3 cm (Fig. 2), whereas the left one measured 2 × 0.5 × 0.3 cm. Histologically, the lesions consisted of adipose tissue, hair follicles, and exocrine glands, covered by normal skin. Hematoxylin and eosin staining of the tissue demonstrated central islands of chondrocytes within the deep dermis (Fig. 3). The differential diagnosis of such cartilaginous tissue within a congenital lesion could include a type 2 first
Case report of bilateral cervical chondrocutaneous remnants
Fig. 1 A photo showing the bilateral cervical chondrocutaneous remnants.
branchial cleft anomaly or chondrocutaneous remnants within the skin (choristoma). A follow-up visit done 9 months postoperatively revealed the patient to be in good health, with no evidence of complications or recurrence of the lesions.
2. Discussion The most common diagnosis of congenital cystic lesions presenting within the neck is that of the second branchial arch anomalies [8]. Chondrocutaneous remnants, which are also thought to arise from the second arch, are very rare. We searched the English medical literature using the Medline and Pubmed databases from January 1966 through December 2010. Only 7 cases of bilateral cervical chondrocutaneous remnants have been reported [2-7]. In 1988, 7 cases of “cervical skin tags” were reported [7], 2 of which were bilateral branchial remnants. The largest case series of chondrocutaneous remnants was reported by Atlan et al in 1997 [6]. They reviewed 20 cases of cervical tags, of which 17 were true cervical chondrocutaneous branchial remnants. They established an association of these choristomas with other malformations, which was in contrast to the previously published case reports. Only 1 case was bilateral in nature. The most common associated malformations were oral (cleft palate and oronasal reflux), auditory (neurosensory deafness, serous otitis media, and external ear
999
Fig. 3 Pathology image showing the cartilage band (C) beneath the skin epidermis (E) and adipose tissue (A). Hair follicles are also evident (H).
malformations), respiratory (tracheomalacia), gastrointestinal (inguinal hernia), genitourinary (hydronephrosis), and cardiovascular (atrial septal defect) [6]. The fact that these lesions were classified under different names (tragi, wattles, rests, choristomas, vestiges, etc) reflects the poor comprehension of their pathology. Because of their resemblance to accessory tragus, some authors have postulated that the origin of these lesions is the pinna rests of the ear [6,9], whereas others suggested a branchial arch origin [10]. According to Atlan et al, both theories are plausible; however, he preferred to divide chondrocutaneous remnants into 2 subtypes according to their location and the type of cartilage they contain [6].The presence of an elastic cartilage directs one to believe that these lesions are auricular in origin, whereas their lower location below the hyoid bone and their association with hyaline cartilage suggests a second branchial arch origin. The clinical characteristics of cervical chondrocutaneous branchial remnants are as follows: male predominance, presence at birth, and location in the middle or lower third of the neck anterior to the sternocleidomastoid muscle. Their skin is similar to the neck skin. The lesions are painless with no erythema or discharge [2]. The reported high incidence of associated anomalies mandates a meticulous physical examination of patients with chondrocutaneous remnants [4,6]. Cervical and preauricular remnants were in some instances associated with Goldenhar, Treacher-Collins, Nager, and some other well-characterized syndromes [3]. Our case was not associated with other congenital anomalies. The treatment of choice adopted in our case and in all published cases was local excision, with an excellent postoperative outcome.
3. Conclusion Fig. 2
Excised left chondrocutaneous remnant.
Cervical chondrocutaneous remnants are rare entities, and the occurrence of a bilateral case is even rarer. Only 7
1000 bilateral cases were reported in the medical literature, and the current one is to be added. Pediatricians, pediatric surgeons, and pathologists should be aware of this entity because of the risk of associated anomalies that can be detected by a detailed physical examination. Local excision seems to be the best treatment modality.
References [1] Park YW. Evaluation of neck masses in children. Am Fam Physician 1995;51:1904-12. [2] Oztürk H, Ozdemir T, Demirbağ S, et al. Bilateral cervical chondrocutaneous remnants: a case report and review of the literature. Turk J Pediatr 2006;48:175-7.
H.A. Nasser et al. [3] Dayal D, Menon P. Bilateral cervical chondrocutaneous branchial remnants. Indian Pediatr 2008;45:221. [4] Rameh C, Sidani C, Arabi M, et al. Bilateral cervical chondrocutaneous branchial remnants associated with cardiac anomalies. J Otolaryngol 2007;36:E79-E81. [5] Braun H, Hofmann T, Wolfgruber H, et al. Case report of bilateral cervical chondrocutaneous branchial remnants. Int J Pediatr Otorhinolaryngol 2003;67:89-92. [6] Atlan G, Egerszegi EP, Brochu P, et al. Cervical chondrocutaneous branchial remnants. Plast Reconstr Surg 1997;100:32-9. [7] Doi O, Hutson JM, Myers NA, et al. Branchial remnants: a review of 58 cases. J Pediatr Surg 1988;23:789-92. [8] Acierno SP, Waldhausen JH: Congenital cervical cysts, sinuses and fistulae. Otolaryngol Clin North Am 2007;40:161-76, vii-viii. [9] Champion RH, Burton JL, Burns DA. Rook/Wilkinson/Ebling. Textbook of dermatology. 5th ed. Oxford: Blackwell-Scientific; 1994. [10] Bendet E. A wattle (cervical accessory tragus). Otolaryngol Head Neck Surg 1999;121:508-9.