A different type of branchial fistula as part of a branchiootorenal syndrome

Branchial cleft and pouch anomalies in childhood: a report of 50 surgical cases

“…Occasionally, first and second branchial cleft anomalies may be part of branchiootorenal syndrome, which is characterized by branchial arch anomalies, hearing impairment and renal malformations [29]. Very rarely, a primary branchiogenic carcinoma has been reported in literature [30].…”

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

“…5 Basic tests include audiometry, temporal bone CT, and abdominopelvic ultrasonography. 5 Further diagnostic investigations may include chromosomal studies. 3 The management of branchial anomalies is primarily surgical, with the goal of achieving a complete excision…”

“…While cysts may present later in life as vague swellings, sinuses and fistulas may be present at birth and drain intermittently early on. 1,5,6 This drainage may be associated with upper respiratory infections, repeated bouts of which may exacerbate symptoms, including cyst enlargement, abscess formation, and rupture. 1,12 Plain radiographs are not helpful.…”

“…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 “dimple swallow” sign for branchial cleft anomalies

“…4 Occasionally, first and second branchial cleft anomalies may be signs of branchiootorenal syndrome. 5 Although a "dimple swallow" sign can be helpful, suggesting the presence of a branchial cleft anomaly (either sinus or fistula), its major clinical value is that it forewarns the surgeon about potential difficulties of the surgical procedure. It implies a deep connection of the lesion, meaning that the tract is either a complete fistula or a deep sinus tethered to the structures that elevate on swallowing.…”