Coexistence of bilateral first and second branchial arch anomalies

Abstract

SUMMARYBranchial arch anomalies are one of the most common congenital anomalies that are usually unilateral and bilateral presentation is rare. The simultaneous presence of bilateral second branchial arch anomalies along with bilateral first arch anomalies is extremely rare, with only three such cases reported in the literature. We present two non-syndromic cases of coexisting bilateral first and second arch anomalies. Developmental anomalies of the branchial apparatus account for 17% of all paediatric cervical masses and are the most common type of congenital cervical mass. They usually present in the paediatric age group. About 96-97% of these anomalies are unilateral. Bilateral presentation is seen in 2-3% having a strong familial association. Congenital syndromes also have been associated with first and second branchial arch anomalies. Thorough clinical examination and investigations should be done to rule out these syndromes. BACKGROUND

A Bilateral, Non-syndromic, Type III Second Branchial Arch Sinus in a Neonate: a Case Report

“…Another main risk that must be avoided in clinical practice is the possible development of branchial carcinoma in the future (3). Alternative treatment methods such as endoscopic electro-coagulation and chemical cauterization are considered as unsafe in pediatric population (18). In conclusion: -in cases of a bilateral second branchial arch sinus, the branchio-oto-renal (BOR) or branchio-otic (BO) syndromes must be excluded; -ultrasound scan can be used for the thorough evaluation of the sinus anatomic course and the relationship with the adjacent anatomic structures; -prompt diagnosis and early therapeutic intervention, even during neonatal period, ensures an uneventful post-operation course.…”

Coexisting First and Bilateral Second Branchial Fistulas in a Child with Nonfamilial Branchio-Otic Syndrome

“…1,5,7,8 Ultrasonography, contrast-enhanced CT, or contrastenhanced MRI may be useful for characterizing cysts, anatomic relationships, and extensions for presurgical planning, 12 but they confer no added advantage in terms of the surgical procedure itself. 8,13 Bilateral branchial anomalies are more often associated with branchio-oto-renal syndrome or branchio-otic syndrome, 5,13 and they necessitate further testing, including evaluation of the ears and the auditory and renal systems. 5 Basic tests include audiometry, temporal bone CT, and abdominopelvic ultrasonography.…”

“…Even more rare are bilateral second branchial fistulas coexisting with first branchial anomalies; to the best of our knowledge, only 10 such cases have been previously reported in the English-language literature. [1][2][3][4][5][6][7][8] Of these 10 cases, 5 were associated with either branchio-otic syndrome or branchio-oto-renal syndrome; 2 patients had familial branchio-otic syndrome, 5,7 2 had nonfamilial branchio-otic syndrome, 1,4 and 1 had nonfamilial branchio-oto-renal syndrome 3 (table). We describe what we believe is only the 11th case of bilateral second branchial fistulas with coexisting first branchial anomalies, and the third such case associated with branchio-otic syndrome in a patient with no family history of branchial anomalies.…”

“…The usual external approach to a second branchial anomaly involves fishmouth external incisions around the inferiorly located puncta, with additional, wider stepladder incisions superiorly to accommodate deeper dissection through the carotid bifurcation. 1,[6][7][8] Transoral visualization and excision of the pharyngeal origin are necessary for complete excision, 7 although transcervical dissection alone terminating superior to the hyoid bone has also been advocated. 13 The necessity of concomitant tonsillectomy 7 has not been established.…”