Abstract

Fourth branchial pouch anomalies are rare congenital disorders of the neck and are a consequence of abnormal development of the branchial apparatus during embryogenesis. Failure to appropriately recognize these anomalies may result in misdiagnosis, insufficient treatment, and continued recurrence. Here, we present an unique presentation of two cases, describe their diagnosis, clinical course, and management, and review the literature regarding these interesting anomalies.

Highlights

  • The human branchial apparatus, which develops in early gestation, is comprised of six paired mesodermal arches, separated by endodermal and ectodermal invaginations known as pouches and clefts, respectively [1]

  • We present two rare cases of fourth branchial pouch abnormalities, the second of which represents a true, complete fistula, an anomaly rarely reported in the literature

  • Anomalies may be characterized as a fistula, sinus, or cyst: a fistula of branchial origin is composed of remnants of both pouch and cleft, with rupture of the interposed branchial plate; a sinus is a tract that is open to either gut or skin, but not both; a cyst is open to neither [9]

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Summary

Introduction

The human branchial apparatus, which develops in early gestation, is comprised of six paired mesodermal arches, separated by endodermal and ectodermal invaginations known as pouches and clefts, respectively [1]. Remnants of the fourth branchial arch are extremely rare with less than 100 cases reported in the literature [6] and account for 1–4% of all branchial anomalies [7]. These anomalies typically present as recurrent neck infections and/ or abscesses or acute suppurative thyroiditis [5, 8]. We present two rare cases of fourth branchial pouch abnormalities, the second of which represents a true, complete fistula, an anomaly rarely reported in the literature. We have emphasized the diagnosis, clinical course, and management of these interesting anomalies

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