Fourth Branchial Apparatus Research Articles

Endoscopic Dyeing and Plasma Coblation-Assisted Open Neck Surgery for Pyriform Fossa Branchial Apparatus Anomalies in Adults.

Objective: Pyriform fossa (PF) branchial apparatus anomalies (PFBAA) are rare congenital third or fourth branchial apparatus anomalies (TBAA or FBAA). This article summarizes our paradigm in managing this condition by combining endoscopic procedures and open neck surgery. Methods: A retrospective review was undertaken concerning PFBAA cases treated at our tertiary medical institution between July 2020 and November 2023. Data were collected from case records. Three sequential steps were implemented: (1) direct laryngoscopy to identify internal orifice (IO), with injection of methylene blue into it; (2) open neck surgery to resect all inflammatory tissues, focusing on the ligation of the sinus tract out of PF; and (3) plasma coblation of IO mucosa. Results: In total, 7 cases (4 men and 3 women) were included (28-67 years old, median age 53). Presenting symptoms were various, with 6 lesions on the left and 1 on the right side. Preoperative (PO) fiberoptic laryngoscopy identified IO in 6 patients, while PO barium esophageal study identified outflow from PF in 4 patients. A preliminary diagnosis of PFBAA could be established in all cases (2 TBAA and 5 FBAA cases). Direct laryngoscopy after general anesthesia identified IO in all cases (2 on the base of PF and 5 on the apex of PF). All the surgical procedures were successful, with uneventful recovery in all the patients. No postoperative complications were observed. All the patients resumed oral fluid intake after confirmation of no pharyngeal fistula by barium esophageal study on the seventh postoperative day. The duration of follow-up was between 6 and 40 months (with a median duration of 27 months). No recurrence was observed. Conclusion: Open neck surgery, assisted by endoscopic dyeing of sinus tracts and plasma coblation of IO mucosa, is a suitable treatment for PFBAA in adults. This paradigm is effective and safe for senior surgeons.

C-cell differentiation in the wall of an aberrant ultimobranchial sinus in the thyroid gland of an old rat.

In mammals, the thyroid gland possesses two types of endocrine cells, follicular cells and C cells, which have different functions but share a similar endodermal origin (although from different regions of the primitive pharynx). Specifically, follicular cells derive from the ventral pharyngeal floor, while C cells derive from the fourth pair of pharyngeal pouches through the ultimobranchial bodies (UBBs). Disruptions to human midline thyroid morphogenesis are relatively frequent and known as thyroid dysgenesis, which is the leading cause of congenital hypothyroidism. In contrast, fourth branchial apparatus anomalies are very rare clinical entities. The aim of this study was to analyze the morphological features and the immunohistochemical pattern of an aberrant ultimobranchial remnant, align with its persistent contribution to the formation of new C cells. The thyroid gland of an old rat was serially sectioned and immunostained for the following markers: calcitonin, thyroglobulin, cytokeratins, PCNA, P63, E-cadherin, beta-tubulin and CD3. We detected a spontaneous congenital defect in the organogenesis of the UBB in an old rat, giving rise to an 'ultimobranchial sinus', which was accompanied by thymic tissue and an abscess. The epithelium contained basal/stem cells and contributed to the formation of abundant C cells and scarce follicular cells. The ultimobranchial sinus is an exceptional finding for representing the first spontaneous abnormality in the development of UBB reported in rats, and the opportunity to observe sustained C-cell differentiation from stem cells in an old rat. These findings are consistent with a common origin of both C cells and follicular cells from UBB.

Prenatal Diagnosis of Third and Fourth Branchial Apparatus Anomalies: Case Series and Comparison with Lymphatic Malformation.

Third and fourth branchial apparatus anomalies are rare congenital anomalies. The purpose of this study was to investigate imaging features of these lesions on fetal MR imaging in comparison with lymphatic malformations, the major competing differential diagnosis in these cases. A retrospective review of our institutional fetal MR imaging database between 1997 and 2019 resulted in 4 patients with confirmed third and fourth branchial apparatus anomalies and 14 patients with confirmed lymphatic malformations. The imaging features were reviewed by consensus, and the Fisher exact test was used to evaluate statistically significant differences between these 2 populations. Four cases of third and fourth branchial apparatus anomalies were imaged at 29 weeks 1 day (range, 23 weeks 1 day to 33 weeks 4 days). All 4 cases demonstrated unilateral, unilocular cysts without reduced diffusion or hemorrhage and a medially directed beaked contour that tapered between the spine and airway at the level of the piriform sinus. Compared with 14 cases of fetal lymphatic malformations imaged at 27 weeks 6 days (range, 21 weeks 3 days to 34 weeks 6 days), third and fourth branchial apparatus cysts were significantly more likely to be unilocular (P < .005) and to have a medially beaked contour (P < .005). The combination of features of unilateral, unilocular, and medially beaked contour was observed only in the fetuses with third and fourth branchial apparatus cysts (P < .001). The presence of a left-sided unilocular cyst with a medially beaked contour tapering at the level of the piriform sinus suggests the diagnosis of third and fourth branchial apparatus anomaly. Accurate diagnosis in the prenatal period allows proper counseling, genetic work-up, and treatment, potentially sparing patients from recurrent infections and associated morbidity.

Combination Surgical Procedure for Fourth Branchial Anomalies: Operative Technique and Outcomes.

Branchial apparatus anomalies of the fourth cleft are the rarest subtype of anomaly and occasionally present with suppurative thyroiditis or thyroid abscess due to their relationship with the thyroid gland. Surgical approaches vary and some surgeons favor cauterization of associated pyriform sinus tracts alone versus complete surgical excision. Currently, the literature is scarce and there is limited data on surgical outcomes and procedural steps. Here we describe a combination surgical technique for fourth branchial anomalies including: (1) surgical excision of the cyst and any external pit, (2) hemithyroidectomy, and (3) direct laryngoscopy with cauterization of pyriform apex tract, if present. A retrospective review was performed on all patients who underwent surgical excision of fourth branchial apparatus lesions (including fistulae, cysts, and sinus tracts) at an urban pediatric university hospital from 2000 to 2019. Data regarding demographics, medical history, surgical methods, complications, and surgical cure rates were collected. A total of 16 patients (9 female, 7 male) underwent a combination surgical procedure for fourth branchial apparatus lesions. Success rate after primary surgery was 94%. One patient had residual disease requiring re-operation. Two patients had post-operative complications: 1 transient vocal fold paresis and 1 seroma, both managed conservatively. A consensus surgical algorithm was created based on operative steps present in the majority of cases. A combination approach to fourth branchial apparatus lesions-including endoscopic cauterization, external excision, and hemithyroidectomy-is safe and provides a high rate of primary cure. Although less invasive options exist, remnants of the branchial lesion, especially in the thyroid, may remain and cause recurrent issues. Therefore, we advocate for complete surgical excision of this rare developmental anomaly, especially when obvious thyroid involvement exists.

Surgical treatment of fourth branchial apparatus anomalies: a case series study

BackgroundFourth branchial apparatus anomalies, are rare clinical entities, and present as complex cysts, sinuses and fistulae in the neck that can be difficult to manage.MethodsThis is a retrospective review of a series of consecutive patients with fourth branchial apparatus anomalies treated at Department of Otolaryngology Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University, from Apr 2014 to Nov 2019.ResultsTen patients with fourth branchial apparatus anomalies were identified, including 8 patients with fourth branchial fistula, and 2 patients with fourth branchial pouch sinus. There were 6 female patients and 4 male patients. Their age was from 6 years old to 39 years old (average age 20.4 years old, median age was 21 years old). All 8 fistulae were on the left side, while 2 pouch sinuses were both on the right side. Pre-operative examination with fiberoptic laryngoscope, barium swallow X-ray, CT or MRI identified internal orifice at pyriform fossa apex in 8 (80%) patients. All patients underwent challenging surgical resection by the senior author. Intra-operative direct laryngoscope confirmed or identified internal orifice in 9 (90%) patients. The tracts were all followed to the vicinity of inferior cornu of the thyroid cartilage and the cricothyroid space. Complete resection of cervical lesions and their attachment to hypopharynx were achieved in 9 cases. No complication occurred. One recurrence was detected, in the only patient whose internal orifice could not be located pre- or intra-operatively, and the hypopharyngeal attachment could not be removed.ConclusionsDirect laryngoscopy under general anesthesia is a reliable method of diagnosis for the fourth branchial apparatus anomalies. Complete surgical removal of fourth branchial apparatus anomalies, including their hypopharyngeal attachment, is the treatment of choice, and the key to prevent recurrence.

Congenital cutaneous fistula at the sternoclavicular joint – Not a dermoid fistula but the remnant of the fourth branchial (pharyngeal) cleft ?

ObjectivesA fourth branchial pouch remnant is well known as a pyriform sinus fistula. However, there has been no report of a fistula composed of the complete remnant of the fourth branchial apparatus. We experienced patients with a congenital lower neck cutaneous fistula which was thought to be the skin-side remnant of the fourth branchial cleft. Materials and methodsSeven children were referred to our hospital from 2009 to 2015 for the treatment of a cutaneous fistula situated near the sternoclavicular joint. All of them were surgically resected and their pathological characteristics were examined. Clinical charts were retrospectively reviewed. ResultsIn six cases, the left side was affected. All cutaneous fistulas had a small skin orifice near the sternoclavicular joint and they were situated at the anterior edge of the sternocleidomastoid muscle. Abscess formation was seen in four cases. Surgical resection was performed at the age of 6 months to 9 years. These fistulas ran deep into the subcutaneous tissue and had a blind end. Pathological examination showed that the epithelial layer was mainly composed of a stratified squamous epithelium. In two cases the epithelium was composed of ciliated columnar epithelium. Recurrence has not been observed in any of the cases. ConclusionThe seven cases had a common clinical feature and were a definite clinical entity. Judging from the characteristics of our cases and the previous literature, we concluded that this lower neck cutaneous fistula was most likely a congenital skin-side remnant of the fourth branchial cleft.

Branchial fistula arising from pyriform fossa: CT diagnosis of a case and discussion of radiological features

Anomalies of third or fourth branchial apparatus origin are very uncommon and present as recurrent neck infections or thyroiditis with a predominant left-sided involvement. Radiological diagnosis requires a high index of suspicion and is critical for initiation of proper treatment. We describe a case of branchial sinus of pyriform fossa with external fistulization that presented in adulthood and was diagnosed on computed tomographic scan. The radiological features of this rare anomaly are revisited.

Complete Congenital Third Branchial Fistula: A Rare Case Report

Fistulae arising from the second branchial apparatus are the most common anomalies than those arising from the third and fourthbranchial apparatus. Third branchial fistula is extremely rare in occurrence.1 Complete fistulae are uncommon as in the majority of casesthe tracts end blindly. Here, we report a case of complete congenital third branchial fistula on left side of the neck with an internal openingnear left pyriform fossa in a 12-year-old male patient.

Revisiting Imaging Features and the Embryologic Basis of Third and Fourth Branchial Anomalies

There is wide discrepancy between common clinical and radiologic presentations of branchial sinuses arising from the pyriform fossa and the theoretic course of third and fourth branchial arch anomalies. The purpose of this study was to revisit the clinical presentations and imaging features of such anomalies in children. A retrospective review of institutional and diagnostic imaging data bases from 1998 to 2008 for reported cases of third and fourth branchial cleft anomalies was conducted. Clinical presentation, pharyngoscopy results, and imaging features in all the patients were evaluated. Surgical and histopathology correlation in patients who underwent excision of the tract was also obtained. Twenty reported cases described as third or fourth branchial apparatus anomalies were identified. There were 12 females and 8 males with a mean age of 84.6 months. The most common presentation was an inflammatory neck mass (18/20, 90%) almost always involving the thyroid gland. Most lesions were on the left side (16/20, 80%). Pharyngoscopy showed a sinus opening at the piriform fossa in 18/20 (90%) cases. None of the cases followed the classic theoretic pathway of third and fourth arch remnants. Histopathology showed tracts lined with pseudostratified squamous epithelium or ciliated columnar epithelium often associated with inflammatory changes in 17 surgically resected cases. Branchial sinuses arising from the pyriform fossa often present with an inflammatory neck mass involving the thyroid lobe, most often on the left side. Imaging and surgical findings suggest that they arise from the embryonal thymopharyngeal duct of the third branchial pouch, because they do not follow the hypothetic course of third or fourth arch fistulas.

Cervical infection secondary to pyriform sinus fistula of branchial origin

Complete third branchial arch anomalies are rare and have been described only in case reports, affecting mainly children and typically presenting as a cervical inflammatory process. Anomalies of the third and fourth branchial apparatus, though rare, usually present as sinuses/incomplete fistulas of pyriform sinus or recurrent suppurative thyroiditis. A 6-year-old girl presented with a small opening on the left side of her anterior neck, which had been present since birth and was associated with recurrent infection. She had no history of incision and drainage of swelling. Computed tomography with contrast injection into the cervical opening revealed a fistulous tract extending from the cervical neck skin to the pyriform fossa. Complete excision of the fistulous tract and left hemithyroidectomy were performed. There was no recurrence at 22 months of follow-up.

Pyriform sinus malformations: A cadaveric representation

Background/Purpose: The most important aspects in management of pyriform sinus malformations are awareness of the diagnosis, familiarity with the clinical manifestations, and complete surgical excision of the entire tract. Pyriform sinus anomalies are the least common branchial apparatus malformations and present anatomically as sinus tracts with or without cystic dilatation. The clinical presentations can include lateral neck mass, thyroid abscess, suppurative thyroiditis, retropharyngeal abscess, neonatal airway obstruction, and even carcinoma. Recurrent symptoms after surgery suggest incomplete identification and excision of the tract. Methods: Cadaveric dissections were performed to show both the proposed embryologic course and clinical manifestations of third and fourth branchial apparatus pyriform sinus anomalies. Results: Illustrations and digital camera images of the cadaveric models are presented to explain the course of pyriform sinus fistula tracts. Conclusions: The authors discuss 3 case presentations of pyriform sinus anomalies with emphasis on their proposed embryologic origin and anatomic basis for surgical management. Surgical excision is the mainstay of therapy. Understanding the embryologic basis for pyriform sinus malformations aids in recognition of the diagnosis despite the myriad of clinical presentations. Laryngoscopy with sinus cannulation facilitates removal of the entire sinus tract with preservation of the recurrent and superior laryngeal nerves.

Congenital lateral cervical cysts of infancy

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.