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The fourth branchial pouch sinus (FBPS) is a rare translaryngeal anomaly with diverse manifestations, including neonatal stridor and recurrent deep neck infection. Review of the world literature reveals 23 reports of sinuses consistent with fourth pouch origin. We present two additional cases, including the only example of a right-sided FBPS. Retrograde excision, beginning at the piriform apex, ensures complete removal of the tract and protection of the recurrent nerve. The posterior border of the thyroid ala must be resected or retracted for adequate exposure. Failure to remove the translaryngeal portion of the tract almost guarantees recurrence.

A male patient presented at day 3 of life with a small anterior neck mass and mild intermittent upper airway obstruction. Despite intravenous antibiotics, the patient rapidly progressed to impending airway obstruction requiring surgical intervention. The clinical, radiographic, and histologic findings of this extremely rare case of a third branchial anomaly will be presented. The embryologic basis for the connection to the aerodigestive tract and the access provided to gram negative enteric organisms in newborns will be discussed.

Bilateral pharyngeal sinuses were found in a 71-year-old male Caucasian anatomic cadaver. As their ostia opened through the piriform recesses cranio-ventral to the plicae of the internal branches of the superior laryngeal nerves, they were determined to be of third pharyngeal (branchial) pouch origin. A publication review revealed that this instance was the tenth case of third pharyngeal pouch sinuses reported in the English literature, and the third case describing a bilateral occurrence. Histologic sections showed the sinuses were lined with a stratified squamous epithelium that was complexly evaginated on the lateral sinus wall. Despite the tendency to accumulate ingested matter, the sinus epithelium showed no chronic pathology. A review of branchial embryology suggested that the relative rarity of third (and fourth) pharyngeal pouch vestiges may be partly explained by the “ascensus” of the hypoglossal nerve and the aortic arch migrations lateral to the pharyngeal regions derived from these more caudal branchial pouches. However, as the literature of the last two decades has surprisingly revealed reports of 28 cases of fourth pharyngeal pouch sinuses, the third pouch sinuses are now the rarest of the reported endobrachial vestiges.

Branchial abnormalities constitute 32% to 45% of all neck pathologies in children. They may be a part of branchiootorenal (BOR) syndrome, which is characterized by branchial arch anomalies, preauricular pits, hearing impairment, and renal malformations. Typically, the management of a branchial fistula does not necessarily require an extensive diagnostic workup. However, in patients with a branchial fistula associated with external ear anomalies on physical examination and/or a history of hearing loss and a similar history and findings in other family members, an additional workup should be performed to eliminate the possibility of BOR syndrome. The aim of this report is to make pediatric surgeons aware of the BOR syndrome in patients presenting with branchial arch anomalies.

Introduction: Branchial cleft cysts are congenital developmental defects of which second branchial anomalies are the most common type. Most of these anomalies present as a lateral neck mass along anterior border of sternocliedomastoid muscle. Careful examination and proper intervention is needed in some of these cases to avoid unwanted complications or even emergencies. Case presentation: A 5 years old boy was brought by his parents with history of dysphagia and snoring for about one month. There was no other associated history of sore throat or shortness of breath or oral bleeding. During fiberoptic examination we found a pedunculated left nasopharyngeal mass. CT imaging showed a left nasopharyngeal hypodense lesion with no vascular or bony invasion. Excision of the cyst was done via combined transoral/transnasal endoscopic approach. Follow up after eight months showed no evidence of recurrence. Conclusion: Second branchial cleft cysts presenting in the nasopharynx are considered rare presentations of the disease and other differential diagnosis should be always brought in mind. Fiberoptic examination of such cases is mandatory to rule out laryngeal involvement and to predict the extension of the cyst for surgical intervention. Surgical excision through combined transoral/transnasal endoscopic including the tract ligation is the treatment of choice to prevent recurrence and to minimize the occurrence of possible secondary infection of the cyst.

Objective: The objective of this study was to review clinical and embryologic aspects of third and fourth branchial anomalies. Study Design: Retrospective study. Methods: We reviewed the institutional and departmental databases at our institution to identify all cases of third and fourth branchial anomalies encountered from 1992 to 2006. All patient records were examined with respect to demographics, clinical history, and radiologic and pathologic reports. Results: We identified 17 cases of third and fourth branchial anomalies, the largest series of its kind reported to date. The lesions were predominantly left sided, all presenting with neck infection. Fistula formation was iatrogenic, secondary to incision and drainage. Preoperative direct laryngoscopy always revealed a pit within the apex of the piriform fossa. Surgical excision involved ipsilateral thyroidectomy as the lesion passed through the thyroid gland. No lesions following the classical course of a either a third or fourth branchial anomaly were identified. Conclusions: The clinical presentation of branchial sinuses arising from the piriform fossa is more in keeping with derivation from the thymopharyngeal duct (of the third pouch) than the hypothetical course of third and fourth branchial fistulae.

We present a case of recurrent anterior neck abscess due to a congenital fourth branchial anomaly. Pyriform fossa sinus is uncommon and poses a diagnostic and therapeutic challenge. This case reiterates that any child with a recurrent anterior neck mass should undergo thorough clinical and radiological assessments to rule out the possibility of a congenital sinus/fistula.

Objective We sought to review the presentation, evaluation, and treatment of branchial anomalies in the pediatric population and to relate these findings to recurrences and complications. Study Design and Setting We conducted a retrospective study at a tertiary care pediatric hospital. Patients Ninety-seven pediatric patients who were treated for branchial anomalies over a 10-year period were reviewed. Patients were studied if they underwent surgical treatment for the branchial anomaly and had 1 year of postoperative follow-up; 67 children met criteria, and 74 anomalies were studied. Results Patients with cysts presented at a later age than did those with branchial anomaly fistulas or sinus branchial anomalies. 32% of branchial anomalies were previously infected. Of these, 71% had more than one preoperative infection. 18% of the BA were first arch derivatives, 69% were second arch derivatives and 7% were third arch derivatives. There were 22 branchial cysts, 31 branchial sinusies and 16 branchial fistulas. The preoperative and postoperative diagnoses differed in 17 cases. None of the excised specimens that contained a cystic lining recurred; all five recurrences had multiple preoperative infections. Conclusions Recurrence rates are increased when there are multiple preoperative infections and when there is no epithelial lining identified in the specimen.

Branchial anomalies are important lesions to consider in the differential diagnosis of head and neck masses in children. These anomalies are composed of a heterogeneous group of congenital malformations that arise from incomplete obliteration of pharyngeal clefts and pouches during embryogenesis. Although present at birth, many abnormalities do not become evident until later in infancy or childhood. It is common for branchial anomalies to become infected, causing significant morbidity. Surgical removal may be difficult, and inadequate resection of the lesion is likely to cause recurrence. Understanding the embryology and anatomy of these lesions is essential to the provision of adequate therapy.

We present our 10 years experience (1995-2005) of second branchial cleft fistulas and sinuses in the pediatric age group by retrospective analysis of records of 52 patients (with 63 fistulas) whose sinuses or fistulas were excised. It was found to be thrice as common in males as compared to females and was predominantly unilateral. The role of fistulogram and methylene blue dye injection in delineation and complete surgical excision of the tract was evaluated. Twenty-five cases were managed by pre-operative fistulogram and intra-operative dye injections for excision, whereas 38 cases of branchial fistulas and sinuses were excised without fistulogram and dye injection. Fistulogram and dye injection were found to be of no extra help during excision of the tract. There were two recurrences.

Fourth branchial anomalies are rare entities. Despite this, they must be considered when presented with a low lateral neck mass, recurrent lower neck abscesses, or recurrent suppurative thyroiditis. The truly unique presentation of this case highlights the importance of including these anomalies in the differential diagnosis for all neck lesions.

Third and fourth branchial pouch anomalies are rare and usually present as lateral neck masses, abscesses or with acute suppurative thyroiditis. An opening in the piriform sinus can be identified in most cases. We present four cases of fourth branchial pouch sinuses, one of a third branchial cyst and discuss our management. Cannulation of the sinus tract at laryngoscopy, followed by complete surgical excision, via a modified oblique thyrotomy above the cricothyroid joint after detaching the inferior constrictor was used to treat the fourth branchial pouch anomalies. This surgical approach adequately exposes the piriform sinus apex and also affords protection to the recurrent laryngeal nerve. The third pouch cyst and tract were excised at the level of the thyrohyoid membrane. There were no complications or recurrences.

Objective: Anomalies of the fourth branchial arch complex are exceedingly rare, with approximately forty cases reported in the literature since 1972. The authors report experience with six fourth arch anomalies. Methods: Retrospective chart review of six consecutive patients presenting to the pediatric otolaryngology service at a tertiary care center with anomalies referable to the fourth branchial arch. Results: All six patients presented within the first or second decade of life. All six had left-sided disease. Four patients presented with recurrent neck infection, one with asymptomatic cervical masses, and one with a neck mass and respiratory compromise. One patient had prior surgery presented with a recurrence. Diagnosis of fourth arch anomalies was suggested or confirmed by computed tomography and flexible laryngoscopy. Treatment was surgical in five patients; one patient is awaiting surgery. Surgical procedures included resection of the mass and endoscopic cauterization of the inner opening of the cyst. Conclusions: The presentation of a cervical mass, especially with recurrent infections and especially on the left side, in a child in the first or second decade of life heightens suspicion for an anomaly of the fourth branchial arch. Diagnosis can be difficult, but is aided by the use of flexible laryngoscopy, Computed tomography (CT) scanning and ultrasonography. Surgical resection of the cyst and cauterization of its pyriform sinus opening should be undertaken to minimize recurrence.

Branchial anomalies present with a wide range of pathologic characteristics, including cysts, fistulas, and sinuses of the head and neck region. Branchial cysts are most commonly diagnosed during the second through fourth decades of life, while branchial sinuses and fistulas are diagnosed almost exclusively in children with infection episodes. Only rarely has an internal sinus of a third or fourth branchial anomaly manifested in adults as a noninfectious swelling in the neck during swallowing. In this report, we describe our experience treating a 21-year-old man with a left-sided swallowing-induced neck protrusion of 10 years' duration. Findings of physical examination, videolaryngoscopy, and a pharyngoesophagogram confirmed the diagnosis of internal sinus of the pyriform fossa, with uncertain origin of a third or fourth branchial anomaly. The patient underwent regular follow-up as an outpatient and experienced no further infectious episodes.

Since branchial anomalies are developmental disorders, pyriform sinus fistula (PSF) has been considered as a pediatric disease, and most reports of PSF with clinical symptoms have thus been on neonatal and young children. There are only a few case reports worldwide, of PSF found in adult patients. Here, we report a case where the patient’s initial symptom of PSF occurred at the age of 72. Interestingly, papillary thyroid carcinoma (PTC) was concurrently identified on computed tomography during the evaluation for PSF. Chemical cauterization and total thyroidectomy was performed for the treatment of PSF and PTC, respectively. This case report may contribute to increased awareness of PSF in adult patients and the possible coexistence of thyroid cancer in these patients.

We thank Dr Wood and his colleagues for their comments regarding our recently published article titled “Lateral Pharyngeal Diverticulum” and for sharing their information on branchial anomalies.Classically, both third and fourth branchial anomalies arise from the pyriform fossa of the hypopharynx. A third branchial fistula pierces the inferior constrictor muscle medially to the greater cornu of the hyoid bone and then courses laterally to the superior laryngeal nerve. It loops over the hypoglossal nerve, runs inferiorly to the glossopharyngeal nerve, and passes poste-riorly to the internal carotid artery. Then, the fistula opens into the skin over the anterior border of the sternocleidomastoid mus-cle. A third branchial cyst or sinus may be found at any point along this course.

Branchial cleft anomalies comprise a heterogeneous group of congenital malformations derived from incomplete in utero resorption of the pharyngeal clefts and pouches. Fistulae, cysts, sinus, tracts and cartilaginous remnants of the first and second branchial clefts are the most common manifestations. These anomalies are excised to prevent the morbidity of subsequent infection. Complete second arch fistulae are rare and comprise 2% of all branchial anomalies. Only 5 cases of bilateral branchial cleft fistulas have been reported in the English-language literature.

Neck abscess located in or around the thyroid gland should raise the suspicion of acute suppurative thyroiditis, pyriform sinus fistula, a 3rd or 4th branchial cleft anomaly. Differentiating between these entities on a clinical basis is difficult. After treating the initial infection, computed tomography, barium esophagography, ultrasound, and endoscopy can be used in search of the fistulous tract that can be associated with a pyriform sinus fistula or a 3rd or 4th branchial anomaly. We present a case of a pyriform sinus fistula involving the use of oral contrast, combined with computed tomography, to delineate the tract and its surrounding structures. This method, in combination with endoscopy, aided in the removal of this branchial anomaly.

Branchial cleft anomalies are congenital developmental defects that typically present as a soft fluctuant mass or fistulous tract along the anterior border of the sternocleidomastoid muscle. However, branchial anomalies can manifest atypically, presenting diagnostic and therapeutic challenges. Error or delay in diagnosis can lead to complications, recurrences, and even life-threatening emergencies. We describe a case of an infected branchial cleft cyst that progressed to a retropharyngeal abscess in a 5-week-old female patient. The clinical, radiographic, and histologic findings of this rare presentation of branchial cleft cyst are discussed.

Design We examined 3 generations in a family for congenital conductive hearing impairment, dysmorphic small auricles, and lip pits. Setting Department of Otorhinolaryngology, University Hospital Nijmegen, Nijmegen, the Netherlands. Results Seven members of the family had bilateral dysmorphic auricles. Three subjects had either a pit or dimple in the lip. Two subjects had congenital conductive hearing impairment. Conclusion Using gene linkage, we confirmed that these autosomal dominant inherited branchial anomalies present a new separate branchial arch syndrome.

Congenital cranial, cervical, and upper thoracic sinuses are rare conditions that historically have been classified according to their location and/or pathology. However, published reports of bronchogenic or branchial anomalies are on the increase, and the traditional defining characteristics--location and histopathology--are proving to be less reliable. We describe the pathologic and clinical findings of a congenital presternal pedunculated lesion with a sinus, and review the literature to describe its proper classification.

Lateral cervical cysts, sinuses, and fistulas have been described as anomalies of the normal development of the branchial apparatus. Third branchial apparatus anomalies are rare and constitute less than 1% of all such cases. Three cases of third branchial cleft cysts and sinus tracts are presented. Two patients had previously undergone multiple attempts at extirpation. Complete removal of recurrent branchial anomalies is difficult because of scarring and fascial plane disruption. Recurrences were often the result of inadequate excision, possibly of the tract communicating with the piriform sinus. To avoid this we advocate endoscopy prior to initial resection of a suspected branchial cleft anomaly to identify any pharyngeal communication. A combined, simultaneous endoscopic identification of the piriform sinus tract with a lateral external cervical dissection facilitates complete resection. In recurrent cases, wide-field extirpation of the cyst, tract, and scar tissue is necessary to ensure complete removal of the branchial cleft anomaly. A review of the literature and of branchial apparatus embryology is also presented.

Received February 5, 2013 Revised March 7, 2013 Accepted March 8, 2013 Address for correspondence Dongbin Ahn, MD Department of OtolaryngologyHead and Neck Surgery, Kyungpook National University Medical Center, 807 Hoguk-ro, Buk-gu, Daegu 702-210, Korea Tel +82-53-200-5777 Fax +82-53-423-4524 E-mail godlikeu@naver.com Background and ObjectivesZZPyriform sinus fistula with 3 and 4 branchial cleft anomaly is an extremely rare congenital condition that is not very well understood owing to its rarity. The aim of the study was to review our experience with pyriform sinus fistulae to better understand them. Subjects and MethodZZOf 163 patients with branchial anomaly treated at our institute between 1999 and 2011, medical records of 15 patients with pyriform sinus fistula were retrospectively reviewed. ResultsZZOverall, 9.2% had pyriform sinus fistulae and this proportion was higher than that of patients with 1st branchial anomaly. The mean age at the time of initial symptom presentation was 11.9 years. However, five patients (33.3%) were not diagnosed at that time because pyriform sinus fistula was not suspected. Computed tomography (CT) was the most sensitive diagnostic tool in patients suspected to have pyriform sinus fistulae; the sensitivity of CT was 86.7%, whereas that for esophagography was only 20%. Conservative surgical treatment involving cauterization of the opening of the fistula tract was performed in nine patients and showed favorable results with 22.2% of recurrence rate during a mean follow-up period of 23.1 months. In 67.7% of the patients, the causative organisms were bacteria inhabiting human mouth, suggesting that infection source for pyriform sinus fistula may be food contents and discharge of upper aerodigestive tract. ConclusionZZThe incidence of pyriform sinus fistula may be higher than expected. Therefore, the possibility of pyriform sinus fistulae coexisting should be considered while treating young patients with perithyroidal infection. Korean J Otorhinolaryngol-Head Neck Surg 2013;56:154-8

An atypical case of a first branchial cleft presenting with a cutaneous fistula and an epidermoid cyst of the external auditory canal is reported. The relevant embryology of the branchial apparatus is summarized, and variations of first branchial anomalies are discussed. The relationship with a congenital cholesteatoma is discussed.

A 21-year-old woman was referred to our hospital because of proteinuria and mild renal dysfunction. Her serum creatinine concentration was 1.10 mg/dl and estimated glomerular filtration rate was 53.9 ml/min per 1.73 m2. The urinary analysis revealed 2+ protein. Abdominal computerized tomography (CT) showed right renal hypoplasia (Figure 1). In addition, she had mixed hearing disturbance and a history of surgeries for correction of left cervical branchial fistulae and bilateral preauricular pits (Figure 2, left ear). Her temporal bone CT presented bilateral inner ear malformation. On the basis of these findings, she was suspected to have branchio-oto-renal (BOR) syndrome, although she has no family history on renal dysfunction and hearing disturbance. The BOR syndrome is an autosomal dominant disorder, which is characterized by the association of branchial anomalies (preauricular pits and branchial fistulae or cysts), otic anomalies affecting the outer, middle, and/or inner ear, which frequently lead to hearing disturbance (sensorineural, conductive, or mixed), and a wide spectrum of renal anomalies ranging from mild hypoplasia to lethal bilateral renal aplasia. The prevalence is approximately 1 in 40,000. The BOR syndrome is associated with several genetic mutations in EYA1, SIX1, SALL1, and SIX5. Because of disagreement towards conducting gene analysis from the patient's family, we performed gene analysis of only the patient with informed consent. DNA sequencing analysis revealed a heterozygous mutation, c.880 C>T, p.R294X, in exon 10 of the EYA1 gene. The BOR syndrome should be taken into consideration on the differential diagnosis in young adult patients with renal dysfunction and hearing disturbance.

First branchial anomalies embody a small subset of congenital malformations of the head and neck. Classically, they are duplication abnormalities of the external auditory canal (EAC) and can involve the middle ear and parotid gland. Herein, we describe a 5-yearold girl with unilateral microtia, autism, and Cayler cardiofacial syndrome (asymmetric crying facies and congenital heart disease). She also had a congenital pharyngo-oto-cutaneous fistula resulting in high-volume otorrhea of saliva and formula. We present our experience with surgical correction of this unique condition. To our knowledge, this is the first reported case of a congenital pharyngo-oto-cutaneous fistula involving a derangement of all 3 germ layers of the first branchial apparatus.

Objective: To evaluate the role of functional neck dissection for treatment of cervical branchial remnants that are recurrent after previous surgical intervention. Design: A retrospective review was undertaken of six patients undergoing functional neck dissection for excision of recurrent branchial anomalies treated over an 18-year period. Setting: Academic tertiary referral medical center. Participants: Six patients with recurrent branchial cysts, sinuses, or fistulas. Each participant previously had undergone between one and 14 ineffective surgical procedures. Intervention: Each patient was treated by functional neck dissection. Outcome Measures: Clinically noted complications and recurrences. Results: There were no major complications. Minor complications included one postoperative wound infection and one transient spinal accessory nerve paresis. There were no recurrences. Conclusions: Functional neck dissection is a safe and effective procedure for surgical management of recurrent cervical branchial remnants. (Arch Otolaryngol Head Neck Surg. 1994;120:417-421)

A retrospective review was made of the medical records of 42 patients, who had had branchial cyst, sinus, or fistula, and who had been seen and treated at our hospitals. Three of these lesions were considered to have originated from the first branchial cleft, 36 from the second branchial cleft and/or pouch, and 3 from the fourth branchial pouch. There were no sex and side of presentation differences in the second branchial anomalies. However, the first branchial anomalies occurred predominantly in females, and the fourth branchial anomalies were predominantly left-sided. Pathological findings were of squamous epithelium and subepithelial lymphoid follicles in most branchial remnants. Skin, adenexa, and cartilage were observed in two first branchial cleft sinuses (Work's classification Type II). Columnar epithelium (respiratory type) was observed in a second branchial pouch cyst and a second branchial fistula. All the patients with first or fourth branchial anomalies had previous infections with incision and drainage procedures, whereas only two out of 36 patients with second branchial anomalies had had previous incision procedures. All patients after complete removal of branchial anomalies have no recurrence.

Branchial cleft cysts and branchial anomalies develop from the branchial cleft apparatus that persists after fetal development. The most common anatomical site for the occurrence of branchial cleft cysts is in the cervical area, generally anterior to the sternomastoid muscle in the upper or middle portion of the neck. A mediastinal branchial cleft cyst is extremely rare and few cases have been reported. We report the case of branchial cleft cyst found in the anterior mediastinum with literature review.

The fourth branchial pouch sinus is a congenital anomaly which most frequently manifests itself by recurrent episodes of neck abscess or acute suppurative thyroiditis. This lesion usually becomes symptomatic before the age of 10 years and is more common than has previously been suspected. It has been found on the left side in 93% of the 28 cases reported in the English literature. Barium swallow during periods of quiescence and nasopharyngoscopy have frequently been successful in identifying the presence of these embryological remnants. Definitive therapy consists of total excision of the sinus tract, which can be facilitated by direct endoscopic placement of a Fogarty catheter into the sinus lumen before surgical exploration. The embryological basis for the occurrence of these sinuses is discussed.

Branchio-otorenal (BOR) syndrome is an autosomal dominant disorder with incomplete penetrance and variable expression characterized by branchial, otic, and renal anomalies. Mutations in the EYA1 gene have been associated with BOR syndrome. In order to demonstrate the utility and limitations of EYA1 testing, clinical features and test results in our BOR syndrome patients were reviewed. A retrospective chart review was conducted on 8 patients identified with BOR syndrome based on diagnostic criteria described by Chang and Smith et al (2004) and in whom EYA1 testing was completed. The clinical features of EYA1 mutation-positive and EYA1 mutation-negative patients were then compared. EYA1 mutations, S251X and W541X, were identified in 2 of the 8 patients. Features present in both EYA1 mutation-positive patients were hearing loss, external ear anomalies (ie, microtia and simple or cupped ears), and branchial anomalies. These findings were less common in the EYA1 mutation-negative patients: hearing loss 83% (5/6), external ear anomalies 67% (4/6), and branchial anomalies 50% (3/6). Preauricular pits, renal anomalies, inner or middle ear malformations, asymmetric crying facies, and a family history suggestive of BOR syndrome were present in at least one of the EYA1 mutation-positive patients. In the EYA1 mutation-negative patients, 83% (5/6) had a family history suggestive of BOR syndrome, 67% (4/6) had preauricular pits, and 17% (1/6) had asymmetric crying facies. Imaging of the temporal bones and renal ultrasound were completed in 5 of the 6 EYA1 mutation-negative patients. Inner or middle ear malformations and renal anomalies were identified in 80% (4/5) and 60% (3/5), respectively. Preauricular tags were present in 50% (3/6) of the EYA1 mutation-negative patients and none of the patients with identified mutations. Although this is a small study, EYA1 mutations are more likely identified in patients with branchial anomalies. Other common features, including preauricular pits, renal anomalies, and various ear malformations, were not alone highly predictive. Asymmetric crying facies, which was present in 2 of the 8 patients, may also be a more commonly associated feature in BOR syndrome than previously described. This study illustrates the various major and minor phenotypic features reported in individuals with BOR syndrome and the continued challenge in identifying genetic changes responsible for this condition and other forms of syndromic hearing loss.

Second branchial cleft and pouch anomalies are by far the commonest of the branchial anomalies. In most cases, however, there is a unilateral cleft sinus. A complete fistula with external and internal openings is rare. We present a case with a complete branchial fistula in which the tract was followed from the neck to the tonsillar fossa using a combined approach.

Objectives To identify the clinical and anatomical presentations and to discuss the guidelines for surgical management of anomalies of the first branchial cleft. Design Retrospective study. Setting Three tertiary care centers. Patients Thirty-nine patients with first branchial cleft anomalies operated on between 1980 and 1996. Intervention All patients were treated surgically. Complete removal of the lesion required superficial parotidectomy with facial nerve dissection in 36 cases. The relationship of the facial nerve and anomalies is discussed. Results Anatomically, 3 types of first branchial cleft anomalies are identified: fistulas (n=11), sinuses (n=20), and cysts (n=8). Clinically, 3 types of presentation are noted: chronic purulent drainage from the ear (n=12), periauricular swelling in the parotid area (n=18), and abscess or persistent fistula in the neck located above a horizontal plane passing through the hyoid bone (n=21). A membranous attachment between the floor of the external auditory canal and the tympanic membrane was observed in 10% of cases. The facial nerve was located lateral to the anomaly in 39% of cases. Conclusions Before definitive surgery, many patients (n=17) underwent incision and drainage for infection owing to the difficulties in diagnosing this anomaly. Wide exposure is necessary in most cases, and a standard parotidectomy incision allows adequate exposure of the anomaly and preservation of the facial nerve. Complete removal without complications depends on a good understanding of regional embryogenesis, a knowledge of the circumstances surrounding discovery, an awareness of the different anatomical presentations, and a readiness to identify and protect the facial nerve during resection.

First branchial cleft anomalies are rare disorders. A case of a first branchial anomaly with an unusual fistulous tract is presented.The patient was a 6-year-old boy who complained of white gelatinous discharge from his left ear canal and recurrent abscess in the left submandibular region. Otoscopic examination revealed deformity of the tympanic membrane and a downward excavation of the bony external auditory canal at its deepest potion. Hearing was normal and there was no deformity in his left auricle or other parts of the body. The fistulous tract was extirpated. The tract extended upwards from the submandibular wound, passing medially through to the deep parotid gland lobe and opening into the bony external auditory canal. The tract was found to be lined with keratinized squamous epithelium with adnexal structures and accompanied by cartilage at the junction of the auditory canal.In the past literature we could not find a case that had a fistulous opening at the bony external auditory canal and a deformity of the tympanic membrane.We observed collected human fetuses (the fourth to eighth weeks) in order to study these anomalies from the embryological standpoint. We found by chance two other developmental stage fetuses both of which showed duplicated external auditory canal and pinna. After careful comparison with these fetuses, we concluded that the first branchial cleft anomaly with the fistulous tract running through from the submandibular area to the external auditory canal were not derived from these duplication anomalies of the external ear. This type of first branchial cleft anomaly was considered to be closely associated with Reichert's cartilage. In our case we supposed there would be interaction between Reichert's cartilage and the tympanic ring during the latter half of the fetal period.The deformity of the tympanic membrane was believed to be caused by incomplete closure of the foramen of Huschke.

First branchial cleft anomaly is an uncommon clinical problem that can be difficult to diagnose and treacherous to treat. It is generally believed that branchial anomalies arise from incomplete resolution of branchial cleft remnants. They may be a fistulous tract or cystic lesions, and they may be found in all age groups. This article presents three cases of first branchial cleft anomaly and offers an overview of the regional embryology and guidelines for surgical management and facial nerve preservation.

7 cases of isolated branchial anomalies distributed in four consecutive generations of a Central Indian family are described: 6 were males and 1 was female. 3 members had a left-sided branchial sinus, 1 had a right-sided sinus, 2 had bilateral sinuses, and 1 had a right-sided cyst. The family shows that branchial (lateral cervical) cysts and sinuses are inherited as autosomal dominant characters, and that the two anomalies are indistinguishable genetically.

Objective: To report an extremely rare variant of first branchial cleft anomaly. Case report: A-13-year old boy presented with the chief complaint of persistent right aural discharge since infancy. On examination, a 1 mm fistulous opening was present in the floor of cartilaginous portion of external auditory canal. And scanty watery discharge came out through this opening when the patient asked to swallow. Sinogram through discharging meatal opening showed a thick irregular tract about 5 cm length traversing the soft tissue of neck extending from the floor of right external auditory canal to the lateral pharyngeal wall and spillage of contrast into the oral cavity. Conclusion: This was an extremely rare variant of first branchial cleft fistula. Due to the rarity of this disease, clinical misdiagnoses and inappropriate management may lead to recurrence and complications. For better prognosis, appropriate diagnosis and surgical planning are very important. Key words: Objective: To report an extremely rare variant of first branchial cleft anomaly. Case report: A-13-year old boy presented with the chief complaint of persistent right aural discharge since infancy. On examination, a 1 mm fistulous opening was present in the floor of cartilaginous portion of external auditory canal. And scanty watery discharge came out through this opening when the patient asked to swallow. Sinogram through discharging meatal opening showed a thick irregular tract about 5 cm length traversing the soft tissue of neck extending from the floor of right external auditory canal to the lateral pharyngeal wall and spillage of contrast into the oral cavity. Conclusion: This was an extremely rare variant of first branchial cleft fistula. Due to the rarity of this disease, clinical misdiagnoses and inappropriate management may lead to recurrence and complications. For better prognosis, appropriate diagnosis and surgical planning are very important. Key words: First branchial anomaly; branchial cleft fistula; branchial cyst; fistulography. DOI: 10.3329/bjo.v17i1.7633 Bangladesh J Otorhinolaryngol 2011; 17(1): 72-75 . DOI: 10.3329/bjo.v17i1.7633 Bangladesh J Otorhinolaryngol 2011; 17(1): 72-75

The course of a fourth branchial fistula is reviewed. Although no complete fistula has yet been described, the anatomy of such a fistula can be determined from a knowledge of the embryologic development of the brachial region. The fistula must first ascend over the hypoglossal nerve before caudal to the fourth area arterial structures. This description is different from the one commonly recognized by otolaryngologists.

Internal branchial sinuses are rare compared to other branchial anomalies. A case is described in which such a sinus presented as an air-containing inflammatory lateral neck mass in a neonate and evidence is presented to support the theory that this originated from the fourth pharyngeal pouch. Review of six similar cases from the literature shows this to be a well-defined clinical entity.

Most congenital lateral cervical cysts, fistulae, and skin tags are considered to be from the branchial apparatus. This is a 13-year review of 58 patients (with 66 branchial lesions) who were operated on. There were eight simple cysts and six cysts with a fistula; 43 external fistulae with or without an internal opening, and nine skin tags. Eighty-seven percent (39/45) of patients with skin tags and external fistulae were less than 5 years of age at the time of operation. On the other hand, all eight patients with cysts but no fistula were greater than 9 years of age. Eight lesions were considered to be the first branchial remnants, and 44 lesions were suspected to be from the second branchial cleft. One external fistula with an internal communication to the pharynx at the level of thyrohyoid membrane was considered to be a third (or fourth) branchial remnant. The other branchial cyst with thyroid tissue in its wall was suggested to be a fourth branchial remnant. Pathology of the excised lesions showed columnar, squamous, or a mixed epithelium. Lymphoid aggregates were documented in 31 patients. Duration of hospital stay was short, except for four patients with first cleft defects who stayed more than five days. Three of the four recurrent cases were first branchial remnants, including one case with the first operation performed at another hospital. In view of these findings, first branchial remnants must be excised with extra care.

To The Editor .—I was extremely interested in the perspicacious report of Dr. G. C. Robinson and Dr. V. J. Wright on "Sensorineural Hearing Loss and Congenital Heterochromia Iridium" (Amer J Dis Child 116: 106-109 [July] 1968). Dr. Robinson and colleagues have been very seminal contributors, and their acute observations have been of keen interest to this reader. Although, as an oral pathologist I have no direct involvement with this syndrome, nevertheless, syndromes of profound childhood deafness have captured my interest, and I have catalogued over 50 such symptom-complexes. Dr. Robinson and Dr. Wright indicated in their report that this binary complex with auricular appendages had not been previously reported. It would appear, however, that the report by McLaurin et al, "Hereditary Branchial Anomalies and Associated Hearing Impairment" ( Laryngoscope 76 : 1277-1288 [July] 1966), may well illustrate this syndrome. The association of hereditary deafness (autosomal dominant) with pretragal ear pits and

Branchial cleft carcinoma is a clinicopathological entity which has aroused considerable controversy in the literature. The concept of carcinoma arising in an embryonic vestigium in the neck was originated by Volkman1in 1882. Since that time sporadic reports and articles have denied the existence of such an entity. Thirty years ago this condition was thought to be more common than had previously been recognized. In 1928 Carp and Stout2reported 4 cases among a total of 32 cases of "branchial anomalies." The following year McWhorter3described 14 cases of "malignant epithelial tumors of the neck of unknown origin," while Hudson4reported 6 and Reader5reported 1. In 1935 Cryle6added 28 cases of "branchial carcinoma" and Oliver7included 80 cases in his article on carcinoma of branchiogenic origin. However, subsequent case reports have not borne out an increase in the incidence. Martin8