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A 1-week-old female infant presented with a transilluminating neck lump that increased in size with crying. The presumptive diagnosis was lymphatic malformation, but imaging raised the possibility of an abscess or necrotic tumour. A biopsy revealed a likely developmental cyst with local inflammatory change. Microlaryngoscopy revealed a fourth branchial pouch sinus, which was cauterised. The sinus and neck lump resolved without need for further treatment., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

Cervical congenital malformations are relatively common in children. They can also be found in adults. The embryological development of the cervical region is closely related to the branchial clefts. This must be a diagnosis made by elimination; a cervical tumor must evoke the diagnosis of cancer. A cutaneous fistula or a cervical tumor, chronic or recent appearance in an inflammatory context, are the clinical signs. The thyroglossal duct cysts and the second branchial clefts cysts are the most common causes of median and lateral cervical cysts, respectively. Imaging contributes greatly to the orientation and diagnostic evaluation of the extent of the lesions. Treatment is initially based on antibiotic therapy and then on complete surgical excision, away from an infectious episode, the sole guarantee for the absence of local recurrence., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: The presence of a branchial fistula with communication both internally and externally: a 'true' branchial fistula is rare, and may arise in the context of autosomal dominant conditions such as branchiootic syndrome and branchiootorenal syndrome., Study: We discuss the case of a true branchial fistula, which recurred after initial surgical excision, in a patient with branchiootic syndrome. The residual tract was dissected in a second operation through stepladder neck incisions and removed in toto via an intraoral approach. No renal abnormalities were detected on investigation with ultrasound., Discussion: Incomplete excision of a branchial sinus is likely to cause recurrence however intraoperative visualisation of the tract can can sometimes prove challenging. An combined intraoral and external approach aids delineation and tract definition when there is a true branchial fistula and can therefore facilitate a complete excision. Suspicion of an hereditary aetiology should be raised in patients with bilateral or preauricular features, or a positive family history, which may then prompt additional renal and genetic investigation., (Copyright © 2014 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.)

Purpose of Review: Branchial anomalies comprise approximately 20% of pediatric congenital head and neck lesions. This study reviews current literature detailing the diagnosis and management of first, second, third and fourth branchial cysts, sinuses and fistulae., Recent Findings: Branchial anomalies remain classified as first, second, third and fourth cysts, sinuses and fistulae. Management varies on the basis of classification. The imaging study of choice remains controversial. Computed tomography fistulography likely best demonstrates the complete course of the tract if a cutaneous opening is present. Treatment of all lesions has historically been by complete surgical excision of the entire tract. Studies of less invasive procedures for several anomalies are promising including sclerotherapy and endoscopic excision of second branchial cysts, and endoscopic cauterization or sclerotherapy at the piriform opening for third and fourth branchial sinuses. An increased risk of complications in children less than 8 years is reported in children undergoing open excision of third and fourth branchial anomalies., Summary: Branchial anomalies are common congenital pediatric head and neck lesions but are comprised by several diverse anomalies. Treatment must be tailored depending on which branchial arch is involved and whether a cyst mass or sinus/fistula tract is present.

Congenital cervical anomalies are essential to consider in the clinical assessment of head and neck masses in children and adults. These lesions can present as palpable cystic masses, infected masses, draining sinuses, or fistulae. Thyroglossal duct cysts are most common, followed by branchial cleft anomalies and dermoid cysts. Other lesions reviewed include median ectopic thyroid, cervical teratomas, and midline cervical clefts. Appropriate diagnosis and management of these lesions requires a thorough understanding of their embryology and anatomy. Correct diagnosis, resolution of infectious issues before definitive therapy, and complete surgical excision are imperative in the prevention of recurrence., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Objectives: To determine the presentation of third or fourth branchial pouch anomalies in various age groups of children and evaluate endoscopic cauterization as a treatment technique., Design: Retrospective study of patients treated from 2000 to 2009., Setting: Tertiary care children's hospital., Patients: Pediatric patients aged 0 to 18 years (mean age, 5.5 years), including 5 neonates., Interventions: Endoscopic and/or open surgical management of third and fourth branchial pouch anomalies; clinical and endoscopic follow-up., Main Outcome Measures: Absence of clinical recurrence; closure of the sinus tract., Results: Two forms of presentation were identified: a neonatal form, characterized by a voluminous and compressive cervical mass (5 of 20 [25%]) and a childhood form, presenting as a cervical abscess (15 of 20 [75%]). The vast majority of our patients regardless of presentation were treated endoscopically (n = 19), with a success rate of 68% (13 of 19) after 1 procedure, 79% (15 of 19) after 2 procedures, and 89% (17 of 19) after 3 procedures. Neonatal and adult presentations require slightly different therapeutic approaches., Conclusions: Third and fourth branchial pouch anomalies can present in 2 distinct forms: a neonatal form and a childhood form. The endoscopic technique should be the favored approach for both forms: whenever possible, in view of its simplicity, rapidity, and the lack of serious postoperative complications. Recurrences can be treated by repeated cauterization using the same technique, with good long-term outcomes. An age-based management algorithm has been developed.

Congenital midline cervical clefts (CMCC) are rare. There have been approximately 50 reports in the international literature with the majority found in Caucasian females. They consist of a skin tag, mucosal surface and a caudal sinus in the ventral midline of the neck. The embryological cause is disputed, as is the best course of treatment. A case of a 4-month-old Indian boy with a congenital midline cervical cleft is reported. The appropriate investigations are described as well as a new method of correcting the lesion. In addition, the most likely aetiological basis for the condition are discussed.

A fourth branchial pouch sinus is a rare congenital anomaly, which in a 13-year-old girl presented clinically as recurrent deep cervical abscesses. The location of the majority of these anomalies is the left side of the neck (90%). Radiological and endoscopic investigations verified the diagnosis. The internal orifice located at the apex of the pyriform sinus could facilitate contamination by infectious pharyngeal secretions and lead to abscess recurrence. Traditionally, the recommended treatment is radical surgery. It can, however, be technically difficult to excise the whole fistula tract. In this patient we used a non-invasive treatment modality; chemocauterization with 40% trichloroacetic acid (TCA). After three treatments the fistula was closed. To date (month no. 15) there has been no abscess recurrence. TCA chemocauterization seems to be a safe first-line treatment for patients with pyriform sinus fistulas.

The recent advent of endoscopic procedures has compelled both plastic and neck and head surgeons to reconsider the conventional methods by which the excision of cervical congenital cystic is classically achieved.An endoscopic approach for excision of the cervical congenital cystic is described. This procedure is anatomically safe and can be made with minimal morbidity through a small transcervical incision. Both specific instruments and solid anatomical knowledge are necessary to perform a safe and efficient cystic endoscopic excision. The essential surgical steps are as follows: 1. Minimal incision placed in natural cervical wrinkle over the dome of the cyst; 2. Intracystic or extracystic dissection; 3. Identification and protection of the sternocleidomastoid muscle, spinal nerve, hypoglossi nerve, and posterior belly of digastric muscle; 4. Careful dissection of the posterior surface of the cyst, avoiding injury on the carotid vessels and internal jugular vein. Eight patients were operated on with this technique and they were very pleased with postoperative comfort and aesthetic results. Inconspicuous scars and no complications were registered. With advanced endoscopic instruments and the development of new surgical technique and surgeon experience, the endoscopic surgery can be the method of choice in cervical excision of branchiogenic cysts.

The fourth branchial pouch sinus is a very rare developmental anomaly; it usually presents as a recurring abscess in the left side of the neck. A high index of suspicion, combined with Barium swallow and computed tomography scan, aids in diagnosis. A case of branchial pouch anomaly is reported here for its rarity and late presentation. Complete excision of the entire epithelial tract combined with ipsilateral thyroid lobectomy remains the mainstay of management.

Fourth branchial cysts are quite rare. A neonate with a left lateral neck mass and respiratory distress was found to have a fourth branchial cyst, which was diagnosed with computed tomography and endoscopy. The characteristic computed tomography findings included an air-containing neck cyst, which was located at the anteromedial site of the common carotid artery with mediastinal extension. Endoscopic examination revealed an internal opening at the apex of the pyriform sinus, communicating with the cyst. Total excision of the cyst was performed, and the specimen, which showed ciliated columnar epithelium with a subepithelial lymphoid infiltrate, thyroid follicles, and thymic tissue, histologically confirmed the diagnosis.

The etiology of lateral cervical cysts in infancy is complex. The cysts are congenital in origin and are derived from the third or fourth branchial apparatus. The authors describe two cases of congenital lateral cervical cysts with an internal opening in the pyriform sinus and possible origin in the third branchial arch. The embryology, clinical presentation, and operative management of this condition is reviewed.

Between 1986 and 1995, 128 patients were treated for various head and neck congenital malformations at Saint-Luc University Hospital, Louvain. We report three cases of fourth branchial pouch cysts requiring surgical removal. One of these cases presented with a third branchial pouch remnant on the same side and subsequently a fourth branchial pouch sinus. To our knowledge, this is the first case published in the literature. A fourth branchial pouch sinus tract can become manifest clinically by recurrent episodes of neck abscess or acute suppurative thyroiditis (especially in infants). The tract can be identified with a barium swallow during the period of latency and hypopharyngeal endoscopy under general anesthesia. Total excision of the fistula with dissection up to the pyriform sinus with or without a left thyroid gland lobectomy and isthmectomy is the treatment of choice.

A female patient with a preotic fistula caused by a defect in the development of the first branchial arch is described. Repeated surgeries for lateral fistula of the neck brought no success. The pathogenesis of the defect and difficulties in its diagnosis are discussed; the authors claim that fistulography with contrast agents may be effective. Histologic verification is needed in all the cases.

Surgical treatment was performed in 66 patients with lateral cyst and 23 with congenital single brachiogenic cervical fistula. In 14 patients, a cyst was complicated by a cervical phlegmon, 17 had a complete fistula, 6--incomplete one. In cyst diagnosis, the clinical signs and puncture of a cyst is of crucial importance, in fistula diagnosis--its connection with the pharynx--introduction of a dye. Removal of the uncomplicated cyst, spontaneously opened cyst-phlegmon (in 58 patients) and fistula (in 20) was performed with the use of the most cosmetic oblique-transverse incision. A lateral cyst often becomes infected and is subject to removal immediately alter establishment of the diagnosis. It is preferable to operate the patients with a lateral congenital cervical fistula non-complicated by infection when they are under school age.

We report a man who had the branchio-oto-renal (BOR) syndrome with crossed renal ectopia. His three children were born with bilateral renal agenesis and the so-called Potter syndrome. This case illustrates the potential severity of the renal anomalies in the BOR syndrome and the inadequacy of oligohydramnios and maternal serum alpha-fetoprotein as screening methods for renal agenesis. This case also implies strongly the necessity for meticulous search for renal anomalies in individuals with the BOR syndrome and proper counseling regarding the possibility of lethal bilateral renal agenesis.

A 15-year-old girl presented with a cystic swelling since birth behind the ramus of the right mandible and diagnosed clinically as a dermoid cyst. Surgical exploration, however, showed that it was closely related to the external auditory canal, with an extension running medially behind the parotid gland and ending in the bony middle ear. The facial nerve was closely related to the deep part of the cyst. Such an anatomical position indicates that this was a first branchial cleft anomaly. Surgical excision of the cyst was performed.

We describe a case of a fistula from the first branchial cleft. At the otorhinolaryngology clinic of the Faculty of Medicine at the University of Zagreb we have operated on more than 100 cases of congenital abnormalities of the branchial arches. However, it is only recently that we had the first case of fistula from the first branchial cleft. We report the clinical aspects and operative course in detail.

Neck masses are frequently encountered in children. Although they are most often due to past infections, they may be of congenital origin. A neck mass in an adult may also be benign and of congenital origin. Common congenital neck masses include thyroglossal duct cysts, branchial anomalies, cystic hygromas and hemangiomas. Cysts, sinuses and fistulas may arise from the branchial apparatus.

Congenital neck masses--thyroglossal duct cysts (TDC) and branchial cleft cysts (BCC)--are frequently encountered in any pediatric surgical practice. While their diagnosis is usually straightforward, unusual or combined presentations may occur. We report eleven cases of unusual patterns of congenital neck masses in children. Two patients underwent resection of a BCC with a sinus tract extending through the carotid bifurcation. Both patients subsequently presented with a new mass near the previous scar, which was thought to be a recurrence but was found at surgery to have TDCs with sinus tracts extending through the hyoid bone. In one patient, a lateral neck mass (presumed to be a BCC) was found at surgery to be a TDC. In all three cases the diagnoses were confirmed histologically. Eight patients presented with a solitary thyroid nodule. Six of these had intrathyroid branchial cleft remnants, and two had intrathyroid TDCs. The diagnosis became apparent at operation in six patients, while in two it was made by the pathologist after hemithyroidectomy. Ages at presentation ranged from 16 months to 14 years. The embryology of these neck structures is closely related. It should not come as a surprise that errors in their development may occur, at times paralleling the occurrence of intrathyroid location of parathyroid glands. The possibility of an embryologic rest in the neck should therefore be kept in mind with all clinically evident neck masses. TDCs and BCCs may coexist in the same patient. The histologic differentiation may be difficult in the presence of inflammation, but differences in structure are often characteristic.(ABSTRACT TRUNCATED AT 250 WORDS)

The usefulness of computer tomography (CT) in the evaluation of a congenital neck mass has received little attention in the literature. We present the CT findings in two relatively common congenital neck masses - the thyroglossal duct cyst and the branchial cleft cyst. These cysts present a relatively common CT appearance of a mass with an enhancing capsule and a lucent center measuring between 20 and 35 Houndsfield Units. The branchial cleft cyst commonly occurs along the lateral border of the neck, anterior to the sternocleidomastoid muscle, while the thyroglossal duct cyst occurs along the midline of the neck. CT is excellent for determining the location, size, extent and character of these two common congenital neck masses.

Lateral cervical sinuses of fistula are usually derivatives of the branchial clefts and pouches and have a more or less typical course in the neck. We present a patient with an extremely long sinus extending from the left supraclavicular area, via the cheek up to the left infraorbital rim. We could not find a similar report in literature. The diagnostic evaluation as well as the successful surgical removal of the malformation through a skin incision in the neck and a second incision in the sulcus gingivobucalis are described. On the basis of a brief review in the embryology of the congenital anomalies of the neck and face some considerations about the possible origin of the cervico-facial sinuses are discussed.

The diagnosis and operative treatment of congenital cervical fistulae and cysts are in many cases difficult, especially abnormalities of the first branchial cleft and of the sinus cervicalis. In our hospital 90 congenital cervical fistulae and cysts were treated surgically between April 1972 and August 1979, excluding cysts of the thyroid gland, lymphangiomas etc. Four of these children with a median cyst and two children with fistulae of the first branchial cleft had recurrences due to partial obliteration and fibrosis of the ducts following inflammation. Other causes for recurrence were atypical locations of the fistulae and cysts. Therefore it is necessary to operate the congenital fistulae and cysts as early as possible.

An outer rim of lymphoid tissue and an inner epithelial lining of squamous composition form the lateral (branchial) cyst of the neck. According to the particular pattern of keratin polypeptides, branchial mass inner lining is shown to be homologous to upper digestive tract (UDT) squamous epithelia. Furthermore, immunostaining of tissue sections with a polyclonal antibody highly specific for the major acidic UDT keratin 13 demonstrates that the epithelium in question is composed of both basal and normally differentiated, i.e. non-keratinizing suprabasal (spinous) cells. 'Import' into a neck lymph node, and rapid growth of oropharyngeal crypt epithelial cells is suggested to initiate a sequence of events leading to an acquired lymphoepithelial mass that may actually present as a 'branchial cyst'.