Computed Tomography Findings of Complete Branchial Cleft Fistula.

Branchial cleft anomalies are embryonic remnants of the branchial arches and are described as the second most common congenital neck mass. Depending on their extent, these anomalies are classified as a cyst, sinus, or fistula with branchial cysts being the most common. Branchial cysts deriving from the second branchial arch are by far the most common, accounting for approximately 95% of all cases. Complete second branch arch fistulas with both an internal and external opening are a rare variant of this anomaly, and even less have been well-documented on computed tomography (CT) imaging in the literature. We present here a case of a 20-year-old female with CT findings consistent with a complete second branchial arch fistula extending from the tonsillar fossa to the external lateral neck.

[Congenital cysts and fistulas on the neck in adults]. / Kystes et fistules congenitaux du cou chez l'adulte.

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.

Distribution of branchial anomalies in a paediatric Asian population.

INTRODUCTION: The objective of the present study was to review the distribution and incidence of branchial anomalies in an Asian paediatric population and highlight the challenges involved in the diagnosis of branchial anomalies. METHODS: This was a retrospective chart review of all paediatric patients who underwent surgery for branchial anomalies in a tertiary paediatric hospital from August 2007 to November 2012. The clinical notes were correlated with preoperative radiological investigations, intraoperative findings and histology results. Branchial anomalies were classified based on the results of the review. RESULTS: A total of 28 children underwent surgery for 30 branchial anomalies during the review period. Two children had bilateral branchial anomalies requiring excision. Of the 30 branchial anomalies, 7 (23.3%) were first branchial anomalies, 5 (16.7%) were second branchial anomalies, 3 (10.0%) were third branchial anomalies, and 4 (13.3%) were fourth branchial anomalies (one of the four patients with fourth branchial anomalies had bilateral branchial anomalies). In addition, seven children had 8 (26.7%) branchial anomalies that were thought to originate from the pyriform sinus; however, we were unable to determine if these anomalies were from the third or fourth branchial arches. There was inadequate information on the remaining 3 (10.0%) branchial anomalies for classification. CONCLUSION: The incidence of second branchial anomalies appears to be lower in our Asian paediatric population, while that of third and fourth branchial anomalies was higher. Knowledge of embryology and the related anatomy of the branchial apparatus is crucial in the identification of the type of branchial anomaly.

A true branchial fistula in the context of branchiootic syndrome: challenges of diagnosis and management.

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.

Congenital cystic neck masses: embryology and imaging appearances, with clinicopathological correlation.

Congenital cystic masses of the neck are uncommon and can present in any age group. Diagnosis of these lesions can be sometimes challenging. Many of these have characteristic locations and imaging findings. The most common of all congenital cystic neck masses is the thyroglossal duct cyst. The other congenital cystic neck masses are branchial cleft cyst, cystic hygroma (lymphangioma), cervical thymic and bronchogenic cysts, and the floor of the mouth lesions including dermoid and epidermoid cysts. In this review, we illustrate the common congenital cystic neck masses including embryology, clinical findings, imaging features, and histopathological findings.

Current management of congenital branchial cleft cysts, sinuses, and fistulae.

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 cysts, sinuses, and fistulae in pediatric surgery.

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.

Branchial anomalies in children.

BACKGROUND: Branchial cleft anomalies are the second most common head and neck congenital lesions seen in children. Amongst the branchial cleft malformations, second cleft lesions account for 95% of the branchial anomalies. This article analyzes all the cases of branchial cleft anomalies operated on at Great Ormond Street Hospital over the past 10 years. METHODS: All children who underwent surgery for branchial cleft sinus or fistula from January 2000 to December 2010 were included in this study. RESULTS: In this series, we had 80 patients (38 female and 42 male). The age at the time of operation varied from 1 year to 14 years. Amongst this group, 15 patients had first branchial cleft anomaly, 62 had second branchial cleft anomaly and 3 had fourth branchial pouch anomaly. All the first cleft cases were operated on by a superficial parotidectomy approach with facial nerve identification. Complete excision was achieved in all these first cleft cases. In this series of first cleft anomalies, we had one complication (temporary marginal mandibular nerve weakness. In the 62 children with second branchial cleft anomalies, 50 were unilateral and 12 were bilateral. In the vast majority, the tract extended through the carotid bifurcation and extended up to pharyngeal constrictor muscles. Majority of these cases were operated on through an elliptical incision around the external opening. Complete excision was achieved in all second cleft cases except one who required a repeat excision. In this subgroup, we had two complications one patient developed a seroma and one had incomplete excision. The three patients with fourth pouch anomaly were treated with endoscopic assisted monopolar diathermy to the sinus opening with good outcome. CONCLUSION: Branchial anomalies are relatively common in children. There are three distinct types, first cleft, second cleft and fourth pouch anomaly. Correct diagnosis is essential to avoid inadequate surgery and multiple procedures. The surgical approach needs to be tailored to the type of anomaly of origin of the anomaly. Complete excision is essential for good outcomes.

Branchial cyst carcinoma revisited: stem cells, dormancy and malignant transformation.

PURPOSE: Branchial cleft cysts are among the most common causes for a congenital neck mass. Branchial cleft cyst carcinoma (BCCC) is a type of cancer that arises from cells within these cysts. Despite the distinct criteria that have been reported for its diagnosis, BCCC remains a controversial entity. CLINICAL REPORT: We report a case of type I, first BCCC, on a 71-year-old white man. The diagnosis was based on the proposed criteria following lesion history and location, surgical excision, histology, and panendoscopy. DISCUSSION: We argue for the first time the hypothesis that congenital branchial cysts and BCCC tumors may result from progenitor cell rests of the embryological branchial development. After a period of dormancy, these cells could eventually awake and proliferate, thus giving rise to branchial cleft cysts. With the acquirement of mutations due to genomic instability, some clones of these cells could transform to malignant stem cells, thus clinically manifesting as BCCC. CONCLUSIONS: The wide recognition of stem cells and their role in carcinogenesis provides a new context for the etiopathogenesis of controversial and rare entities such as the BCCC.

[Retrospective analysis of the treatment of recurrent congenital fistula or cyst in lateral cervical part].

OBJECTIVE: To analysis the therapeutic procedure on the recurrent congenital fistula or cyst in lateral cervical part. METHOD: Thirty-nine cases with recurrent congenital fistula or cyst in lateral cervical part were enrolled in this study including 12 cases from the first branchial cleft, 6 from the second branchial cleft and 21 from the third branchial cleft. RESULT: All the cases underwent fistula or cyst excision for 2 to 5 times in their whole therapeutic process, not counting the incision and drainage. During 9 months to 17 years follow-up, fistula or cyst in 6 cases relapsed,including 1 fistula from the first branchial cleft,3 fistulae from the second branchial cleft, 1 fistula and 1 cyst from the third branchial cleft, respectively. One case with recurrent fistula from the first branchial cleft was diagnosed temporal verrucous carcinoma six months after the third fistula excision operation and died one year after the forth operation probably due to the intracranial metastasis of temporal bone verrucous carcinoma. In two cases, the fistulae went through the thyroid gland to the piriform fossa and both the fistulae and part of the thyroid glands were resected. In the patients whose inner orificium fistulae were found and ligated effectively,no recurrence occurred during the followed-up period. CONCLUSION: The key point to cure the recurrent congenital fistula or cyst in lateral cervical part lies in proper occasion of operation, stain tracing in operation and reasonable program of operation.

Neonatal vs delayed-onset fourth branchial pouch anomalies: therapeutic implications.

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.

Pattern of congenital neck masses in a Kenyan paediatric population.

BACKGROUND: The distribution of congenital neck masses varies between countries and is important in diagnosis and treatment modalities. Data from Africa is scarce, and altogether absent from Kenya. OBJECTIVE: To describe the pattern of congenital masses in a Kenyan paediatric population. SET UP: Kenyatta National Hospital, Nairobi Kenya. STUDY DESIGN: Prospective descriptive cross-sectional. METHODOLOGY: Children aged 15 years and below with neck masses who presented to various surgical clinics and wards at the Kenyatta National Hospital between December 2006 and April 2007 were included in the study. They were examined for age, gender, type and location of the neck mass. Mode of investigation and final diagnosis were recorded. Data was analyzed by using statistical package for social sciences. Descriptive statistics were applied to determine means, frequencies and modes. Ninety-five percent confidence interval was used and p value of 0.05 taken as significant. Data are presented in form of tables. RESULTS: Two hundred and thirty-five children (129 male) with neck masses were examined. Fifty-one (22%) of these masses were congenital. Thyroglossal duct cysts were the most common (29%) followed by cystic hygromas (21%) and branchial cleft cysts (20%). Fifty-one percent of the masses were present at birth. The midline was the most common location (31.4%) followed by anterior border of sternocleidomastoid (27.5%) and submandibular region (19.6%). Ultrasound was the commonest diagnostic investigation. CONCLUSION: Congenital defects constitute an important differential diagnosis for paediatric neck masses in Kenya. Thyroglossal duct cysts, cystic hygromas and branchial cleft cysts are the most prevalent occurring most commonly in the midline and anterior border of sternocleidomastoid muscle. An understanding of the distribution of these masses improves diagnosis, preoperative decision making and their overall management.

Congenital neck masses.

Congenital neck lesions reflect abnormal embryogenesis in head and neck development. A thorough knowledge of embryology and anatomy is critical in the diagnosis and treatment of these lesions. The appropriate diagnosis of these lesions is necessary to provide appropriate treatment and long-term follow up, because some of these lesions may undergo malignant transformation or be harbingers of malignant disease.

Congenital neck masses: a descriptive retrospective study of 252 cases.

PURPOSE: This retrospective study was conducted to analyze congenital neck masses in Jordanians compared with international findings. PATIENTS AND METHODS: The records of patients with congenital neck masses between 1991 and 2002 were retrieved from the Department of Pathology, Jordan University of Science and Technology, and analyzed for age, gender, location, clinical features, and treatment. RESULTS: Within the 12-year period of the study, a total of 2,063 neck mass lesions were found; of these, 252 (12%) were congenital masses. These cases were distributed into 166 (66%) midline, 55 (22%) lateral, and 31 (12%) entire neck masses. The most frequent mass was thyroglossal duct cyst (fistulas) (53%), followed by cysts (fistulas) of the branchial apparatus (22%), dermoid cysts (11%), hemangiomas (7%), and 1ymphangiomas (6%). The majority of branchial arch anomalies (85%) were of the second arch. The mean age of patients was 16 years, with the greatest number of cases (38%) in the first decade. The male-to-female ratio was 1:1.2, with most lesions affecting females. The majority of lesions presented with painless neck swelling, and all lesions were surgically excised. CONCLUSIONS: The overall frequency and age distribution of congenital neck masses in northern Jordanians are similar to international findings. However, the gender distribution and relative frequency of individual types of neck masses are somewhat different from those in other countries. These differences may be attributed to genetic and geographic differences.

Anomalías congénitas del aparato branquial: estudio de imágenes / Congenital anomalies of the branchial apparatus: imaging study

: The branchial apparatus consists of six arches that are separated from each other by branchial clefts and pharyngeal pouches. Its development is complex and multiple congenital defects can occur; including branchial cleft cysts, sinuses and fistulas, thymic cysts, aberrant thymic tissue and parathyroid cysts. The majority arises from the second branchial arch and they correspond to vestigial remnants from incomplete obliteration of the branchial apparatus or buried epithelial cell rests. Imaging studies are very helpful in the diagnosis and characterization of these anomalies, and require a good understanding of the embryogenesis. Considering the anatomical location and the radiological appearance the diagnosis could be precised in most cases. The purpose of this presentation is to show the appearance of some of the most frequent congenital anomalies of the branchial apparatus using different imaging modalities and emphasizing the normal embryologic development.

El aparato branquial, formado por seis arcos separados por hendiduras extemas y bolsas faríngeas en su parte interna, tiene un complejo desarrollo del cual pueden derivar anomalías como senos, fístulas o quistes y alteraciones del timo o glándula paratiroi-des. La mayoría de las malformaciones derivan del segundo componente branquial. Los estudios de imagen son útiles para el diagnóstico y caracterización de estas lesiones que será más exacta con un adecuado conocimiento del desarrollo embriológico. El objetivo de esta presentación es revisar la embriología y el aspecto más común en imágenes de algunas de estas anomalías.