A Rare Case of Right Fourth Branchial Fistula: A Diagnostic Challenge.

Fourth branchial anomalies are extremely rare and are often misdiagnosed. A recurrent history of anterior neck discharges or infections since childhood should raise a high clinical suspicion of branchial fistula and necessitate a thorough clinical, endoscopic, and radiological evaluation. We report a rare case of right-sided fourth branchial fistula in a middle-aged lady who was referred to us for recurrent right neck infections since childhood and had received multiple courses of antibiotics and drainage of abscesses. Despite previous negative barium swallow and fistulogram results, the diagnosis of the branchial fistula was made clinically with the spillage of methylene blue dye into the apex of the right pyriform sinus from flexible nasopharyngolaryngoscopy in the clinic after the injection of dye through the fistula opening at the neck. Finally, another barium swallow study and computed tomography scan were conducted, revealing the fistula tract. Complete surgical excision of the fistula tract was then performed with no evidence of recurrence after six months of follow-up.

Management of first branchial anomalies in children: 20 years of experience.

PURPOSE: Branchial cleft anomalies (BCAs) are common pediatric head and neck lesions; however, only 1-4% involve the first branchial cleft. The rare occurrence of first BCAs, their presentation at a young age, and the possible facial nerve involvement make diagnosis and treatment challenging. METHODS: A retrospective chart review was conducted for children diagnosed with their first BCA between 2000 and 2020. Data on demographics, presenting symptoms, physical findings, imaging features, previous surgery, and treatment outcomes were collected and analyzed. RESULTS: The cohort included 17 patients with a median age of 5 years at presentation. Seven (41%) had undergone previous surgical intervention before definitive surgery. Eight were classified as Work Type II anomalies, and nine as Work Type I. Sixteen patients (94%) underwent definitive surgical excision at a median age of 6.9. A parotid approach was used in 10 (62%), with dissection of the mass from the facial nerve, and a retro-auricular or end-aural approach was used in 6 (38%). Complete excision was achieved in 14/16 patients (88%). Three patients had transient facial nerve paresis postoperatively. Recurrence was noted in 3/16 patients (18%). Enhancement in imaging was positively correlated with post-operative complications (R = 0.463, P = 0.018). CONCLUSIONS: First, BCA poses a diagnostic and surgical challenge; thus, definitive surgical treatment is often delayed. The surgical approach should be tailored to the type of anomaly (Work type I or II) and possible facial nerve involvement. Risk factors for post-operative complications are a history of recurrent infections and previous surgical interventions. The presence of contrast enhancement in preoperative imaging should alert surgeons to perioperative challenges and the risk of post-operative complications.

Cisto branquial da 4ª fenda ­ relato de caso / Branchial cyst 4th cleft ­ case report

Introdução: Os cistos branquiais são tumores congênitos laterais, resultantes de defeitos de desenvolvimento embrionário que afetam os arcos branquiais.1 As anomalias congênitas cervicais são mais comumente diagnosticadas nos primeiros anos de vida. Objetivo: Informar, discutir e analisar condutas para tratamento desse tipo de cisto congênito. Método:Relato de caso e análise de dados, diagnósticos e conduta baseada na literatura referente a Cisto Branquial da 4° Fenda. Resultados:Os cistos podem se manifestar tardiamente, mas as fístulas são, quase sempre, diagnosticadas ao nascimento ou na infância.São extremamente raros, estima-se que 95% das anomalias das fendas branquiais sejam da 2ª fenda; das 5% restantes, quase todas são da 1ª ou 3ª fenda.O diagnóstico é primariamente clínico, mas a ultrassonografia pode auxiliar no diagnóstico diferencial de um cisto branquial. O tratamento das anomalias branquiais é a excisão cirúrgica. Lactente sexo feminino, 9 meses de idade em acompanhamento de cisto branquial com conduta conservadora. O surgimento da massa se deu logo ao nascimento, havendo drenagem espontânea do cisto para o esôfago alguns dias depois. Após nove meses paciente retorna devido aumento progressivo da lesão que correlacionando com exame físico, exames de imagem levaram ao diagnóstico de cisto de 4º fenda branquial. Realizada cirurgia para remoção de Cisto juntamente com retirada de lobo esquerdo da tireoide (tireoidectomia parcial). Conclusão:Após a exerese da lesão paciente evoluiu satisfatoriamente sendo encaminhado para enfermaria e posteriormente alta com acompanhamento ambulatorial com pediatra geral.

Introduction: Branchial cysts are congenital tumors, resulting from embryonic defects that affect the branchial arches. Congenital cervical abnormalities are usually diagnosed in the first years of life. Objective: To inform, discuss and analyze treatment approaches for this type of congenital cyst. Method: Case report and analysis of data, diagnoses and approaches based on the literature addressing Fourth Branchial Cleft Cysts. Results: Cysts can manifest late, but fistulas are almost always diagnosed at birth or in childhood. They are extremely rare: it is estimated that 95% of branchial cleft anomalies involve the second cleft; of the remaining 5%, almost all arise from the first and third clefts. There are about 45 cases of fourth cleft cysts reported in the literature. The diagnosis is primarily clinical, but the ultrasound can be used for the differential diagnosis of a branchial cyst. Computed tomography will show air-fluid level in the anterior portion of the neck, in front of the thyroid and trachea, which may compress the trachea, causing respiratory distress in childhood. The treatment of branchial anomalies is surgical excision. A 9-month old female patient was being followed up after conservative treatment of a cervical mass (branchial cyst). The cyst appeared immediately after birth, but there was there was spontaneous drainage of the cyst into the esophagus a few days later. After nine months, the patient returned due to a progressive increase of the lesion, which, after physical examination and imaging exams, led to the diagnosis of a fourth branchial cleft cyst. A surgical procedure was performed to remove the cyst along with the left thyroid lobe (partial thyroidectomy). Conclusion: After the excision of the lesion, the patient made a good recovery. She was then referred to the infirmary and later discharged with outpatient follow-up by a general pediatrician.

Sistrunk centennial: Evolution of a classic operation.

OBJECTIVES/HYPOTHESIS: To trace the evolution of the Sistrunk procedure. STUDY DESIGN: Historical review. METHODS: We reviewed historical materials in the English, French, and German literature including journal articles, book chapters, genealogical sources, and obituaries to identify the emerging concepts leading to the Sistrunk operation. These included references to the embryology of the thyroid and of the branchial apparatus as well as incremental advances in the surgical management of midline congenital cervical cysts and fistulae. PubMed, the Google Internet search engine, Archive.org, and Google Translate were primary investigational tools. RESULTS: Understanding of human embryology evolved rapidly in the mid and late 1800s. Discovery of the thyroglossal duct and its role in thyroid development led to improvements in existing surgery for thyroglossal duct remnants. Sistrunk was aware of the anatomy and histopathology of these remnants and used this knowledge to refine existing surgical approaches. CONCLUSIONS: The elements of thyroglossal duct remnant surgery existed for decades before Sistrunk popularized his modified approach to dissection of the suprahyoid portion of the tract. His innovation and his clear description of the operation made it accessible to surgeons around the world. LEVEL OF EVIDENCE: NA Laryngoscope, 130:E45-E47, 2020.

Branchial cleft cysts: a pictorial review.

Cystic masses of the neck may represent a wide variety of pathological conditions. Among these, branchial anomalies are the commonest paediatric congenital lesions of the head and neck. This article intends to review this condition under the same headings, as well as facilitate their diagnosis with the help of appropriate radiological findings of patients diagnosed with these anomalies. In order to make an accurate diagnosis, it is imperative to have an idea about the possible differentials, and the normal embryological development of the branchial arches, as well as their anomalies, in the form of sinuses, fistulae, and cysts, with their features and subtypes. The medical history of the patient and clinical manifestations helps in suspecting branchial cleft cysts; confirmatory imaging modalities include computed tomography, magnetic resonance imaging, ultrasonography, and fine-needle aspiration. The mainstay of management is usually by surgical excision. The location, clinical picture, and radiological correlation, along with a strong degree of suspicion for the condition, facilitates the diagnosis of this relatively common embryological anomaly.

Branchial anomalies in children: A report of 105 surgical cases.

BACKGROUND: Branchial anomalies (BAs) account for 20% of all congenital masses in children. We sought to review the incidence of involvement of individual anomalies, diagnostic methods, surgical treatment, and complications of BAs in children. In addition, we also classified our study and analyzed a congenital lower neck cutaneous fistula near the sternoclavicular joint that was thought to be the skin-side remnant of the fourth BAs. METHODS: We conducted a retrospective analysis of 105 children who were referred to our hospital from June 2009 to December 2016 for the treatment of BAs. RESULTS: In this series, there were 51 males and 54 females. The age at the time of operation varied from 19 days to 13 years, and the mean age was 4.5 years. A total of 33 (31.4%) cases presented with first BAs, 13 (12.4%) presented with second BAs, and 59 (56.2%) presented with third and fourth BAs, including 6 cases of congenital lower neck cutaneous fistula. Fistulectomy under general anesthesia was performed on all of them. For postoperative complications, 2 cases had temporary facial paralysis, 1 case had permanent facial paralysis, 4 cases had temporary recurrent laryngeal nerve injury. Recurrence occurred in 2 patients with first BAs after medium follow-up time of 3.6 years (6 months-8 years). CONCLUSIONS: BAs are common congenital head and neck lesions in children, and there are four distinct types (first, second, third and fourth anomalies). The incidence of third and fourth BAs in Asia maybe higher when compared with literature reports, second BAs seem rare in this population, but more research is needed to confirm this perspective. Diagnosis is not difficult with a proper knowledge of the anatomy of the BAs. The surgical procedures should be tailored depending on the various types, and complete excision of the fistula is the key to prevent recurrence.

Second Branchial Anomalies: A Study of 94 Cases.

Ninety-four patients with second branchial anomalies were retrospectively analysed at a tertiary care centre from January 2006 to September 2016 to determine the demographical data and management. Branchial sinus and fistula presented earlier as compared to branchial cyst. The mean age at presentation in case of branchial sinuses, fistulae and cysts was 5.07, 5.79 and 7.31 years respectively. There was preponderance in males as compared to females, more so in bilateral cases. Male to female sex ratio was 2.91:1. The branchial fistulae were the most common type of lesions, followed by the branchial sinuses. The branchial anomalies were more on the right side (65.96%) probably due to right handedness of the population. Only eight patients (8.51%) had bilateral anomalies. Four patients had familial association, it was seen in bilateral cases and they presented earlier than unilateral cases. Early and complete surgical excision is the treatment of choice. Preoperative sinogram/fistulogram and intraoperative methylene blue dye injection is not mandatory for excision of a branchial sinus/fistula. Post-operative wound infection was the most common complication (4.25%).

Recurrent pyriform sinus fistula successfully treated by endoscopic Glubran 2 sealing: A rare case and literature review.

OBJECTIVES: The authors present the case of a 3-year-old girl with a history of complicated surgery for removing a third branchial cleft fistula. METHODS: An endoscopic approach using N-butyl-2-acrylate and metacrilosisolfolane glue (GLUBRAN 2) to seal the fistula was performed. RESULTS: The clinical and radiological 6-year follow-up confirmed the absence of the fistulous orifice and the persistence of scar due to previous open-neck surgical procedures. CONCLUSION: endoscopic Glubran 2 sealing has been an effective treatment procedure for branchial fistula.

[Clinical characteristics and surgical management in patients with third and fourth branchial anomalies].

Objective:To analysize the clinical characteristics as well as the effect and methods of the surgical treatment in patiets with the third and fourth branchial anomalies.Method:The clinical data of 25 patients diagnosed as third and fourth branchial cleft fistula by pathological method were analyzed retrospectively.Two of 25 patients had undergone fistulectomy simply.Based on the embryologicc and anatomic features of branchial anomalies,23 of 25 patients had received different types of selective neck dissection.All of lesions were confirmed as branchial cleft fistula by pathology.All patients were received the examinations of Esophagus myelography,MRI and CT preoperatively.Result:The features of the third and the fourth bianchial fistula were as following:most patients suffered from recurrent neck abscess and had undergone incision and drainage. Esophagus myelography and CT were important auxiliary examination for branchial anomalies.No recurrent and complications were found in all patients by using treatment of selective neck dissection (23/25 cases) and fistulectomy simply(2/25 cases) within 12 to 36 months following-up,postoperatively.Conclusion:Branchial anomalies is characterized by recurrent acute abscess,acute thyroiditis or fistula secretion inferior to neck.Complete removal of branchial lesions and inflammatory granuloma using selective neck dissection is a safty and effective treatment for recurrent branchial anomalies.

Thyroidal abscesses in third and fourth branchial anomalies: not only a paediatric diagnosis.

INTRODUCTION: Acute suppurative thyroiditis and recurrent abscess formation due to third and fourth branchial anomalies typically present in children. However, thyroid abscesses in branchial anomalies may occur in adulthood as well. Failure to recognize and delayed drainage of a neck abscess may lead to a fulminant life-threatening outcome. METHODS: This is a retrospective case series. The study group comprised all patients presenting over a 12-month period from January to December 2012 with thyroid abscesses and a branchial cleft anomaly in two centres, one adult and the other paediatric. Patient demographics, clinical presentation, imaging, surgical management, definitive histology and outcomes were documented. RESULTS: Five patients were identified with a history of thyroid abscesses. Only one was a child (aged 9 years) with the other four being adults (aged 20, 34, 37 and 41 years). All patients had third or fourth left branchial cleft anomalies, presenting as suppurative thyroiditis with a left-sided thyroid abscess. Management options ranged from abscess drainage on initial presentation, primary thyroid lobectomy or delayed thyroid lobectomy following abscess drainage. CONCLUSION: Acute suppurative thyroidits and thyroid abscesses is not just a paediatric diagnosis but may present at any age. In both children and adults, a thyroid abscess almost always arises from branchial cleft anomalies.

Second branchial cleft fistulae: patient characteristics and surgical outcome.

BACKGROUNDS: Second branchial cleft anomalies predispose to recurrent infections, and surgical resection is recommended as the treatment of choice. There is no clear consensus regarding the timing or surgical technique in the operative treatment of these anomalies. Our aim was to compare the effect of age and operative techniques to patient characteristics and treatment outcome. METHODS: A retrospective study of pediatric patients treated for second branchial sinuses or fistulae during 1998-2012 at two departments in our academic tertiary care referral center. Comparison of patient characteristics, preoperative investigations, surgical techniques and postoperative sequelae. RESULTS: Our data is based on 68 patients, the largest series in the literature. One-fourth (24%) of patients had any infectious symptoms prior to operative treatment. Patient demographics, preoperative investigations, use of methylene blue, or tonsillectomy had no effect on the surgical outcome. There were no re-operations due to residual disease. Three complications were observed postoperatively. CONCLUSIONS: Our patient series of second branchial cleft sinuses/fistulae is the largest so far and enables analyses of patient characteristics and surgical outcomes more reliably than previously. Preoperative symptoms are infrequent and mild. There was no difference in clinical outcome between the observed departments. Performing ipsilateral tonsillectomy gave no outcome benefits. The operation may be delayed to an age of approximately three years when anesthesiological risks are and possible harms are best avoided. Considering postoperative pain and risk of postoperative hemorrhage a routine tonsillectomy should not be included to the operative treatment of second branchial cleft fistulae.

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.