Diagnosis and treatment of branchial cleft anomalies in UKMMC: a 10-year retrospective study

Branchial cleft anomalies result from abnormal persistence of branchial apparatus, which is located at the lateral part of the neck. These occur due to failure of obliteration of the branchial apparatus during embryonic development. Differential diagnoses of lateral neck mass are salivary gland or neurogenic neoplasms, paragangliomas, adenopathies, cystic hygroma or cystic metastasis from squamous cell carcinoma or thyroid papillary carcinoma. Clinically, a branchial cyst is smooth, round, fluctuant and non-tender, and usually occurs over the upper part of the neck, anterior to the sternocleidomastoid muscle. Sometimes, it may present as infected cyst (or abscess), a sinus or fistula. Surgical excision is the definitive treatment for branchial anomalies. The objective of the work was to study the demographic data, clinical presentation, definite diagnostic workup and treatment of patients diagnosed with branchial anomalies. This is a retrospective study of 26 patients who were diagnosed with branchial anomalies (branchial cyst and fistula), of which only 12 patients had data available between July 1999 and June 2009 at the Otorhinolaryngology, Head and Neck Surgery, Universiti Kebangsaan Malaysia Medical Centre. Twelve cases of branchial anomalies were seen, in which 10 patients had second branchial cyst anomalies, 1 had third branchial fistula and 1 had bilateral branchial lesion. There were seven females and five males. The age of the patients varied over a wide range (4–44 years), but the majority of the patients were in their second and third decade of life. All branchial anomalies occurred at the classical site; eight patients had left-sided neck lesion. Correct clinical diagnosis was made only in five patients (41.6 %). All patients underwent surgical excision with no reported recurrence. Branchial anomalies are frequently forgotten in the differential diagnosis of lateral neck swelling. Diagnosis is usually delayed, leading to improper treatment. The diagnosis of patients who present with lateral neck cystic swelling with or without episodes of recurrent neck abscess should be considered with a high suspicion for branchial anomalies. FNA cytology is a good investigative tool in reaching toward a diagnosis of branchial lesion, with the concurrent assistance of radiological modalities. Surgical excision is the gold standard treatment of lesions of branchial anomalies.     

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.     

Cervical thymic remnants in children

OBJECTIVE: Define the clinical presentation, diagnostic value of preoperative imaging, surgical management, and outcomes of treatment of congenital cervical thymic remnants in children. DESIGN: Retrospective cohort. SETTING: Single tertiary care institution. PATIENTS: 20 children who underwent excision of cervical thymic remnant, 1975-2006. MAIN OUTCOMES MEASURED: Utility of preoperative imaging to diagnose cervical thymic anomalies; success of surgical treatment of cervical thymic remnants. RESULTS: A total of 20 children were identified, with an average age of 6.98+/-5.63 years. All ectopic thymus tissue was found in the embryonic distribution area associated with the third branchial pouch. Fourteen patients underwent excision of a cystic ectopic thymus. Four of these patients exhibited lesions isolated to the cervical region, and 10 patients displayed lesions involving cervicomediastinal areas. Six patients underwent excision of solid ectopic cervical thymus, and each of these was an unanticipated mass encountered during surgical dissection for other procedures. 83% of patients with solid ectopic cervical thymus presented at age 3 or younger. Physical exam and preoperative imaging correctly diagnosed thymic remnants in 15% patients. Resection of thymic remnants was successful in all patients, and there were no recurrences. CONCLUSIONS: Though rare, thymic remnants should be considered in the differential diagnosis of masses presenting in locations associated with derivatives of the third branchial pouch. Though preoperative imaging is helpful in identifying the extent of these lesions, congenital thymic remnants prove difficult to diagnosis radiologically. Surgical excision is the diagnostic and therapeutic treatment of choice in the management of cervical thymic remnants.     

Branchial cleft (pouch) anomalies: a review of 42 cases

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.     

先天性耳颈瘘管的手术治疗

目的：进一步认识先天性耳颈瘘管的解剖异常特征和临床表现,并探讨该病的外科治疗方法.方法：回顾性分析21例先天性耳颈瘘管患者的临床资料,均采用手术治疗.结果：21例患者疗效满意,无腮腺瘘、面瘫及外耳道狭窄等并发症发生.结论：对该病的充分认识有利于早期诊断,完整地切除是治愈该病的惟一选择.腮腺的充分暴露及面神经的解剖与保护是完整切除瘘管及避免并发症发生的关键因素.     

Anatomical variations of the facial nerve in first branchial cleft anomalies

OBJECTIVE: To review our experience with branchial cleft anomalies, with special attention to their subtypes and anatomical relationship to the facial nerve. STUDY DESIGN: Case series. SETTING: Tertiary care center. PATIENTS: Ten patients who underwent resection for anomalies of the first branchial cleft, with at least 1 year of follow-up, were included in the study. The data from all cases were collected in a prospective fashion, including immediate postoperative diagrams. INTERVENTION: Complete resection of the branchial cleft anomaly was performed in all cases. Wide exposure of the facial nerve was achieved using a modified Blair incision and superficial parotidectomy. Facial nerve monitoring was used in every case. MAIN OUTCOME MEASURES: The primary outcome measurements were facial nerve function and incidence of recurrence after resection of the branchial cleft anomaly. RESULTS: Ten patients, 6 females and 4 males,with a mean age of 9 years at presentation, were treated by the senior author (P.J.K.) between 1989 and 2001. The lesions were characterized as sinus tracts (n = 5), fistulous tracts (n = 3), and cysts (n = 2). Seven lesions were medial to the facial nerve, 2 were lateral to the facial nerve, and 1 was between branches of the facial nerve. There were no complications related to facial nerve paresis or paralysis, and none of the patients has had a recurrence. CONCLUSIONS: The successful treatment of branchial cleft anomalies requires a complete resection. A safe complete resection requires a full exposure of the facial nerve, as the lesions can be variably associated with the nerve.     

Cervical chondrocutaneous branchial remnants – Report of 17 cases

Background/purposeCervical chondrocutaneous branchial remnants are congenital, benign and rare neck masses. These anomalies are limited in the literature, reported mostly as case reports.Cervical chondrocutaneous branchial remnant is always present at birth, and the lesion is usually unilateral. Understanding and treatment of cervical chondrocutaneous branchial remnants requires knowledge of the related embryology.MethodsFrom January 2005 to December 2008, 17 patients with mean age of 32 months (range from 2 months to 15 years) with CCBRs were treated at the Division of Pediatric Surgery, Department of Plastic and Reconstructive Surgery and Burns at the Institute for Mother and Child Health Care, Belgrade, Serbia. The following objections were recorded: sex, lesion side, surgical data, associated malformations and pathohistology findings.ResultsThere were 7 females and 10 males, 4 with bilateral presences. Five children had associated anomalies, as follows: vesicoureteral reflux, atrial and ventricular septal defect, ventricular septal defect, branchiootorenal syndrome and preauricular sinus.There was a positive family history in one patient. Fifteen patients (88%) were treated with complete surgical excision and no connections with deep underlying structures of the neck were found. There were no complications at surgery. No recurrence was found during follow-up. Histopathology analysis revealed both, hyaline and elastic cartilage.ConclusionCervical chondrocutaneous branchial remnants are rare anomalies arising from branchial arch, probably originally from remnants of first or second arch. Surgical excision is the treatment of choice. From our experience, we suggest surgical treatment early in childhood because of esthetic reason, simplicity of the intervention and low complication rate. Also, the abdominal ultrasound and cardiac examination is recommended because of associated anomalies.     

74例鳃裂囊肿及瘘管临床分析

目的 总结74例鳃裂囊肿及瘘管的临床特点,探讨其诊疗方法。 方法 收集2010年1月至2019年9月收治的74例鳃裂囊肿和鳃裂瘘患者的临床资料并结合文献进行回顾性分析。 结果 本组患者中有50例为第二鳃裂病变,13例为第一鳃裂病变,10例为第三鳃裂病变,1例为第四鳃裂病变,于我院初次手术时术前误诊率为10.81%。所有患者均采取手术治疗,随访3个月~10年。除3例失访患者外,67例患者一次性治愈,3例患者术后复发,1例患者术后出现永久性面神经麻痹。 结论 先天性鳃裂囊肿及瘘管临床表现多样,鉴别诊断较为复杂,应合理选用多种检查手段,术前准确评估,制定个体化的手术方案彻底切除病灶是首选治疗方法。     

Branchial sinus of the piriform fossa: reappraisal of third and fourth branchial anomalies

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.     

87例鳃裂畸形临床特征分析

目的 总结鳃裂畸形患者的临床特点,探讨其临床诊疗方案。方法 收集2000年1月—2022年9月确诊为鳃裂畸形的87例患者的临床资料并结合文献进行回顾性分析。结果 本组患者中,第一鳃裂畸形患者10例,第二鳃裂畸形患者52例,第三鳃裂畸形患者20例,第四鳃裂畸形患者5例。15例患者有过相关颈部手术史。所有患者均采取手术治疗,仅1例出现暂时性面神经麻痹,术后3个月恢复。随访6个月至16年,81例患者完成术后随访。6例患者术后出现复发,再次行颈部肿物切除术+等离子梨状窝瘘口封闭术,随访7~8年均未再复发。结论 先天性鳃裂畸形诊断较为困难且相对容易复发,术前结合多种检查可提高诊断准确性,手术彻底切除病灶是首选治疗方案,急性感染期患者先予以颈部清创,待稳定期再行手术切除可以降低术后复发率。     

第四鳃裂畸形的诊断和手术治疗

目的:分析第四鳃裂畸形的临床特点,探讨其诊断和手术治疗方法。方法:对10例第四鳃裂畸形患者的临床资料进行回顾性分析,并结合文献对诊断、鉴别诊断及手术治疗方法进行探讨;全部患者均经手术彻底切除病变组织,妥善处理内窦口。结果:随访1～3年,10例均无复发。1例术后即出现声音低沉及饮水呛咳,2周后恢复正常;1例术后即出现伸舌左偏,3个月后复诊恢复正常。结论:反复发作的颈前区中下部的颈深部脓肿及慢性感染窦道,应考虑第四鳃裂畸形的诊断。颈部增强CT扫描及钡餐检查可辅助诊断,病理检查可确诊。手术彻底切除病变组织是治疗第四鳃裂畸形的有效方法,明确内窦道的走行特点及其感染扩散途径,采用选择性颈清扫术的手术原则,是保证彻底切除病变的关键。     

Complete branchial fistula. Case report and review of the literature.

Branchial anomalies, a result of aberrant embryonic development, are rarely seen in clinical practice. Lesions of the second branchial pouch commonly present as a neck lump or discharging sinus that may be complicated by infection. Clinical examination often reveals the lesion to be related to the junction of the upper two thirds and the lower one third of the sternocleidomastoid muscle. Branchial fistulas often present as a discharging sinus in the neck with the fistula tract extending upward within the deep neck tissue for a variable distance. A complete branchial fistula is one that has a defined internal opening in the tonsillar area and an external opening at the skin overlying the sternocleidomastoid muscle at the junction of the upper two thirds and the lower one third of the muscle. The incidence of such lesions is extremely rare. Surgical excision is the treatment of choice for branchial anomalies. We present the case of a patient who presented with a complete branchial fistula and discuss the clinical presentation and surgical management of such lesions, with a review of the relevant literature.     

The double auditory meatus--a rare first branchial cleft anomaly: clinical presentation and treatment

OBJECTIVE: To discuss the embryology, classification, clinical experience with, and management of first branchial cleft anomalies. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Patients with a first branchial cleft anomaly. INTERVENTION: Surgery or revision surgery. MAIN OUTCOME MEASURES: Classifications according to Work, Olsen, Chilla; previous diagnostic and therapeutic pitfalls; outcome of intervention (including facial nerve function). RESULTS: Between 1984 and 1999, first branchial cleft anomalies were diagnosed in 18 patients. Surgical treatment was the treatment of choice. The authors' approach in Work type I and type 2 lesions is described, and surgical aspects of revision surgery are discussed. The importance of early establishment of the relationship of the anomaly to the facial nerve is stressed. In 8 patients, previous surgical attempts had been undertaken without establishment of the diagnosis first. After intervention, the outcome was favorable. CONCLUSIONS: First branchial cleft anomalies occur sporadically in ordinary clinical practice. They may go unrecognized or may be mistaken for tumors or other inflammatory lesions of in the periauricular region. However, the distinct clinical features, which can be derived from embryologic development, usually lead to the correct diagnosis. This avoids both treatment delay and eventual failure.     

Second branchial cleft fistula: is fistulogram necessary for complete excision

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.     

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.     

先天性第四鳃裂畸形的诊断和治疗

目的 探讨先天性第四鳃裂畸形(congenital fourth branchial anomaly,CFBA)的解剖学特点、临床表现、诊断和外科处理原则.方法 回顾性分析8例CFBA患者的临床资料,年龄27～300个月(中位年龄114个月);男4例,女4例;初治3例,复发5例;病变均位于左颈,其中囊肿型1例,窦道型3例(均为内瘘口),瘘管型4例;3例表现为急性甲状腺炎,4例表现为颈深部脓肿,1例表现为颈部肿块.结果 术前检查包括食管吞钡8例次、直接喉镜4例次、CT 5例次、MRI 5例次.急性期患者采取充分引流、控制感染;静止期患者行病灶完整切除+喉返神经解剖+甲状腺腺叶部分切除,对复发病例采用择区性颈清扫术根除瘢痕、炎性肉芽和病变组织.术后1例患者切口局部感染,经换药后愈合;1例患者出现暂时性声带麻痹,1个月后完全恢复.患者随访13～42个月,中位随访时间21个月,未见复发.结论 CFBA走行与喉返神经和甲状腺关系密切.食管吞钡、直接喉镜检查最具诊断价值,CT和MRI有助于明确诊断.治疗原则为感染静止期行喉返神经解剖和甲状腺腺叶部分切除,必要时切除部分甲状软骨翼板以减少并发症和预防复发,复发病例可采用择区性颈清扫术治疗.     

Management of anomalies of the third and fourth branchial pouches

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.     

First branchial cleft cyst excision with electrophysiological facial nerve localization

OBJECTIVE: To assess the safety and efficacy of surgical excision of selected first branchial cleft cysts using electrophysiological rather than anatomical location of the facial nerve. DESIGN: Retrospective review of consecutive surgical procedures by a single surgeon, using a consistent technique during a 9-year period. SETTING: Tertiary pediatric medical center. PATIENTS: Eleven children with first branchial cleft cysts. INTERVENTIONS: Selected first branchial cleft cysts were removed using a smaller surgical approach than that generally advocated. The facial nerve was localized using electrophysiological means rather than superficial parotidectomy and identification of the nerve trunk and branches. MAIN OUTCOME MEASURES: Successful removal of the lesion, avoidance of facial nerve injury, incidence of Fry syndrome, and cosmesis. RESULTS: Eleven patients underwent surgical excision of first branchial cleft cysts during a 9-year period. Ten lesions were removed without the need for anatomical localization of the facial nerve trunk. There was no facial weakness, recurrence of the lesions, or Fry syndrome during a follow-up of 6 months to 7 years. Cosmesis was superior. CONCLUSION: Electrophysiological location of the facial nerve may, in the appropriate setting, replace anatomical localization for first branchial cleft cysts that are (1) superior to the stylomastoid foramen and (2) not previously infected or surgically violated.     

Brown tumors of the jaws associated with primary or secondary hyperparathyroidism. A clinical study and review of the literature

PURPOSE: The aim of this article is to present the development of brown tumors in the jaws as a definite feature of hyperparathyroidism (HPT), whether primary or secondary. PATIENTS AND METHODS: The study included 2 cases of primary HPT and 3 cases of secondary HPT associated with brown tumors in the jaws. The patients presented in our clinic at the "G. Papanikolaou" Hospital in Thessaloniki for treatment of their disease. The patients were 4 women and 1 man, and patient's ages ranged from 21 to 76 years. Four cases of the brown tumors involved the mandible, and 1 case involved the maxilla and the maxillary sinus. The surgical excision of bone lesions and treatment of primary or secondary HPT were performed on the patients in our study. RESULTS: Patient 1 (primary HPT, caused by an adenoma of the right parathyroid glands) was treated with surgical removal of the maxillary lesion and excision of the adenoma. Three years later, the patient is free of the disease, with no recurrence of the brown tumor. Patient 2 (primary HPT, caused by an adenoma of the right parathyroid glands) was treated with excision of the adenoma. One year later, a second bone lesion developed in the mandible, and 3(1/2) of the fourth parathyroid (right) glands was removed. The patient is being observed. The other 3 patients with brown tumors associated with secondary HPT were treated with excision of the bone lesions; HPT was treated with hemodialysis (2 patients) and renal transplantation (1 patient). For the patients treated with hemodialysis, the disease is under control and there is no recurrence of the brown tumors. The patient who underwent transplantation is free of the disease 7 years later. CONCLUSIONS: Primary or secondary HPT may be recognized by the presence of an osteolytic lesion with giant cells, a condition referred as "brown tumor." The most useful therapy for patients with brown tumors is surgical excision of bone lesions and therapy (surgical or medical) for primary or secondary HPT.