Journal of Dermatology for Physician Assistants

The official journal of the Society of Dermatology Physician Assistants

Congenital Cartilaginous Rest of the Neck: A Case Report and Topic Overview

By Peter A. Young, MPAS, and Emily Green, MD

CASE REPORT
A 14-year old male patient was referred to our outpatient dermatology service by his pediatric Nurse Practitioner (NP) for a firm, flesh-colored nodule on the left side of his anterior neck. (Figure 1) It had been present since birth and was not growing in size. He denied pain, tenderness, itch, and purulence–however, the mass rubbed daily on his shirt collar, and peers at school teased him about it, causing anxiety. The patient and his mother requested that the mass be removed. Eighteen months prior to presentation at dermatology, his pediatric NP ordered a soft tissue neck computed tomography (CT) with contrast, which revealed “a focal, nonspecific skin thickening in the left supraclavicular neck, with a prominent vein at this location. No worrisome underlying mass [was] identified.” The patient’s medical history included anxiety, attention deficit hyperactivity disorder (ADHD), mild depression, and enlarged vestibular aqueducts.

In joint evaluation with an attending dermatologist, we determined pilomatricoma was most likely, and the patient and parent consented to our recommendation of simple excision under local anesthesia.

During excision, a firm, white subcutaneous nodule was identified and carefully dissected with blunt-tipped curved scissors. At its base, the nodule felt “tethered” by a dense fibrous band, which was cut with some difficulty using scissors, freeing the nodule. Although the mass was confined to the dermis (superficial, far from the carotid) and in the anterior triangle (far from the spinal accessory nerve), extreme caution was exercised due to the radiologist’s note of a prominent vein near the site.

After staining with hematoxylin and eosin (H&E) stain, histologic sections showed dermal cartilage and absence of a cyst. (Figure 2) In conjunction with the patient’s history of a nodule along the lower lateral neck present since birth, these findings were compatible with the diagnosis of a branchial cartilaginous remnant (also called a cervical cartilaginous rest or congenital cartilaginous rest of the neck [CCRN].

DISCUSSION
Branchial cleft remnants are the second most common congenital lesions of the head and neck in children (next to thyroglossal duct anomalies). They are anomalies of embryologic development: remnants of the branchial arch, arising from incomplete involution of branchial cleft structures during embryogenesis. Present at birth as asymptomatic skin-colored subcutaneous nodules, they are typically located over the lower anterior border of the sternocleidomastoid muscle (SCM). They may be attached to the underlying fascia by a fibrous band, which can be safely transected if excision is desired. Once excised, recurrence is uncommon. There is a broad differential diagnosis for congenital neck masses, and branchio-oto-renal (BOR) and branchio-oculo-facial (BOF) syndromes should be suspected when a patient presents with preauricular pits and branchial anomalies. These syndromes are typically associated with hearing loss, ear malformations, and renal anomalies in the BOR syndrome.

CONCLUSION
The keys to clinically distinguishing a CCRN from other common childhood neck masses are: location (off midline, over the anterior SCM), history (present at birth, stable in size) and palpation (“springy” feeling, absence of a cyst or fistula).

Our patient returned for suture removal 12 days after excision, with no complications. Pathology results were discussed with the patient and mother, and he was re-examined. He did not have preauricular pits, nor other facial abnormalities. His mother stated that when his enlarged vestibular aqueducts were previously diagnosed by his otolaryngologist, he had undergone tests for renal abnormalities, which were negative.

KEY POINTS:
1. CCRNs are a rare subtype of branchial cleft remnant that can be identified clinically by presence at birth, location over the anterior SCM, and “springy” feeling on palpation.
2. Because of their superficial location in the anterior triangle (away from vulnerable anatomy, such as the spinal accessory nerve), CCRNs can easily and safely be treated with careful excision.
3. Referral to an otolaryngologist to evaluate for other congenital malformations may be necessary.

References
1. Bolognia, Jean, MD. (2018) Dermatology, 4th Ed.
2. Adams, Ashock. (2015 Dec. 10). Branchial Cleft Anomalies: A Pictorial Review. Insights Imaging. Retrieved from: ncbi.nlm.nih.gov/pmc/articles/PMC4729717/
3. Blattner, C.M. (2016). Congenital Cartilaginous Rest of the Neck in a Boy. UC Davis Dermatology Online Journal. Retrieved from: escholarship.org/uc/item/43b4k7j6
4. Anouchka, H.C. Branchial Cleft Cyst. (2019 Apr. 21). StatPearls. Retrieved from: ncbi.nlm.nih.gov/books/NBK499914/

Peter A. Young, MPAS, is from The Permanente Medical Group in Sacramento, California. Emily A. Green, MD, is from Golden State Dermatology, in Walnut Creek, California.

Acknowledgment: The principal author would like to acknowledge DelRay Maughan, MD, a now retired otolaryngologist. DelRay’s vast knowledge and dedication to alleviating human suffering was clear in his patient care and instruction. The positive effects on younger clinicians of his mentorship will echo through generations long after his own practice has concluded.

Disclosures: The authors have disclosed no potential conflicts of interest, financial or otherwise, relating to the content of this article.

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