Fourth branchial pouch sinus: diagnosis and treatment

The fourth branchial pouch sinus (FBPS) is a rare translaryngeal anomaly with diverse manifestations, including neonatal stridor and recurrent deep neck infection. Review of the world literature reveals 23 reports of sinuses consistent with fourth pouch origin. We present two additional cases, including the only example of a right-sided FBPS. Retrograde excision, beginning at the piriform apex, ensures complete removal of the tract and protection of the recurrent nerve. The posterior border of the thyroid ala must be resected or retracted for adequate exposure. Failure to remove the translaryngeal portion of the tract almost guarantees recurrence.     

An unusual presentation of a fourth pharyngeal arch (branchial cleft) sinus

Fourth pharyngeal arch (branchial cleft) sinuses and fistulas are a rare cause of recurrent neck abscesses presenting in the first 2 decades of life. Because they are uncommon, the diagnosis and subsequent treatment can be delayed, even with proper radiographic imaging and clinical evaluation. This case illustrates a life-threatening presentation of a ruptured fourth branchial cleft sinus with extravasation into the mediastinum, chest, and abdomen.     

Treatment of asymptomatic preauricular sinuses: challenging conventional wisdom

Congenital preauricular sinuses must not be considered trivial lesions. They can and should be differentiated from branchial cleft cysts. Cure is achieved only through complete excision of the sinus tract and its associated cysts. Total extirpation of the sinus tract is difficult in the presence of infection. The embryoanatomy and treatment of congenital preauricular sinus tracts as well as our experience with 6 patients are reported. We recommend excision of asymptomatic congenital preauricular sinus tracts.     

Ten years of experience with third and fourth branchial remnants

Background: Third and fourth branchial remnants may result in cysts and abscesses that are in close contact with the thyroid gland. These anomalies are rare and often present diagnostic and therapeutic challenges. Methods: The charts of patients diagnosed with a branchial anomaly between July 1991 and July 2001 at the Montreal Children's Hospital were reviewed. All cases of third and fourth branchial remnants or pyriform sinus fistulae were identified. Clinical presentation, imaging, treatment, and outcome were recorded. Results: Eight patients with a third or fourth branchial anomaly were identified and ranged in age from birth to 13 years. All anomalies were left sided. Presenting symptoms consisted of an asymptomatic cervical mass (n = 1), an infected mass (n = 5), neonatal respiratory distress (n = 1), and 1 incidental cyst found on magnetic resonance imaging. Ultrasonography was useful in suggesting the diagnosis in 7 cases. Barium swallow was performed in 3 patients with 2 positive results. Pharyngoscopy results showed the internal opening in 2 of 7 patients. A portion of the thyroid gland was resected in 6 patients. One patient has not yet undergone a definitive procedure. There was 1 recurrence in a patient whose pathology did not confirm a branchial remnant. Conclusions: The diagnosis and management of pyriform sinus anomalies are challenging. Ultrasound scan, computed tomography scan, barium swallow, and pharyngoscopy are all useful. The portion of thyroid involved in the fistula must be excised en bloc with the inflammatory mass, and the tract should be ligated at the level of the pharynx to minimize recurrence.     

Congenital cervical salivary fistula

A study of 35 lesions diagnosed as branchial cleft sinuses or fistulas identified a homogeneous subgroup characterized by ectopic salivary tissue, draining serous or mucoid fluid through a short sinus tract to the base of the neck anteriorly. These lesions should more properly be called congenital cervical salivary fistulas, since they may not arise from the branchial apparatus but rather from the embryonic placodal duct.     

Simultaneous branchial cleft and thyroid disorders may present a management challenge

BACKGROUND: Cysts, sinuses or abscesses arising from second, third or fourth branchial cleft remnants may lie either within the body of, or in close proximity to the thyroid gland. Given their infrequent nature they may pose both diagnostic and management challenges for the treating surgeon when they occur in association with thyroid disorders. METHODS: This is a case series. All patients with concomitant thyroid disorders and a branchial cleft anomaly treated in the University of Sydney Endocrine Surgical Unit in the 10-year period 1994-2003 comprised the study group. Patient demographics, clinical presentation, imaging, surgical management, definitive histology and outcomes were documented. RESULTS: Six patients were identified with an age range of 3-76 years and a male : female ratio of 1:5. Five branchial cleft anomalies were left sided, one was right sided. Two patients had second cleft anomalies, both of which were initially thought to represent metastatic lymph nodes in association with thyroid cancer. A further two patients had third cleft abnormalities presenting as suppurative thyroiditis. The final two patients had fourth cleft abnormalities causing intraoperative management problems. CONCLUSIONS: Branchial cleft remnants and anomalies are rare but may occur in association with thyroid disease. They may pose a diagnostic and management dilemma either preoperatively, when mistaken for metastatic thyroid cancer, or intraoperatively when mistaken for a thyroid nodule.     

Atypical first branchial cleft fistula: A case report

IntroductionFirst branchial cleft anomalies (FBCA) are rare. They have an estimated incidence of 1 in 100,000. Type I are those that embryologically duplicate the membrane (cutaneous) external auditory canal.The aim of this case is to describe an unusual path of a type II first branchial cleft fistula tract in a 3 years old child and its surgical management in the academic hospital of Casablanca.Case presentationThis case is about a 3 year old girl who presented to the Ear Nose Throat (ENT) consultation for recurrent right lateral cervical infection. Clinical examination found an unsightly scar attached to an orifice giving pus located near the right mandibular angle suggesting type II first branchial cleft anomaly.Surgical excision was performed under general anesthesia by the superficial parotidectomy approach, the facial nerve was identified and preserved. The fistula cord was dissected and followed, it went under the facial nerve and the parorid gland to end under the digastric muscle where we tied it up. The postoperative check-up did not show any complications. The follow-up period was 12 months; the clinical examination did not find any sign of recurrence.ConclusionFirst branchial cleft fistula are rare and can be in form of cyst or fistula. Its management is surgical excision keeping the tract cyst of the fistula intact with facial nerve preservation.     

First branchial cleft anomalies involving the tympanic membrane and middle ear

First branchial cleft anomalies may involve the tympanic membrane or middle ear. A complete otologic examination must be performed in any patient with a suspected first branchial cleft anomaly. A surgeon treating such a defect must be prepared to perform both the excision of the lesion and reconstructive otologic surgery.     

Complex branchial fistula: a variant arch anomaly.

A 5-year-old boy presented with an infected left-sided branchial fistula. Despite antibiotic treatment and repeated excision of the fistula, purulent discharge from the wound persisted. Three-dimensional computed tomography (3D CT) reconstruction greatly facilitated the diagnosis and management of this case by showing the course of the fistulous tract. The complexity of the tract suggests that this represents a variant arch anomaly because it contains features of first, second, third, and fourth arch remnants.     

A third branchial pouch cyst presenting as stridor in a child

We present a rare case of a third branchial pouch cyst in an 18-month-old child, presenting with stridor and a lateral cervical cystic mass. Differences in the anatomical course of third and fourth branchial cysts, and histological differences between branchial pouch and branchial cleft cysts are discussed.     

Simultaneous carotid endarterectomy and excision of ipsilateral branchial cleft fistula--a case report

Branchial cleft sinuses are congenital anomalies that usually present in childhood or early adulthood. It is uncommon that such a sinus will remain asymptomatic to the sixth decade of life. This case describes the presence of a second branchial cleft sinus in the setting of carotid artery disease, where both were treated surgically simultaneously without complication.     

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.     

First branchial cleft sinus in hemifacial microsomia

In this paper we present a case of first branchial cleft sinus in hemifacial microsomia. An external opening was placed about 2 cm infero-posterior to the microtic ear remnant. The literature is reviewed and discussed.     

Branchial remnants: a review of 58 cases

Most congenital lateral cervical cysts, fistulae, and skin tags are considered to be from the branchial apparatus. This is a 13-year review of 58 patients (with 66 branchial lesions) who were operated on. There were eight simple cysts and six cysts with a fistula; 43 external fistulae with or without an internal opening, and nine skin tags. Eighty-seven percent (39/45) of patients with skin tags and external fistulae were less than 5 years of age at the time of operation. On the other hand, all eight patients with cysts but no fistula were greater than 9 years of age. Eight lesions were considered to be the first branchial remnants, and 44 lesions were suspected to be from the second branchial cleft. One external fistula with an internal communication to the pharynx at the level of thyrohyoid membrane was considered to be a third (or fourth) branchial remnant. The other branchial cyst with thyroid tissue in its wall was suggested to be a fourth branchial remnant. Pathology of the excised lesions showed columnar, squamous, or a mixed epithelium. Lymphoid aggregates were documented in 31 patients. Duration of hospital stay was short, except for four patients with first cleft defects who stayed more than five days. Three of the four recurrent cases were first branchial remnants, including one case with the first operation performed at another hospital. In view of these findings, first branchial remnants must be excised with extra care.     

The operative treatment of renal lithiasis

A boy of five with probable luetic parents, retarded mental development and bilateral branchial cleft sinuses.Attached to the sinus on the right side was a salivary glandular structure which discharged its secretion through the sinus to the cutaneous surface. This salivary structure responded to the stimulus of food mastication. Total extripation resulted in a permanent cure.     

Cystic metastases of the neck from occult thyroid adenocarcinoma.

In 9 of 118 patients with differentiated thyroid carcinomas, a solitary cystic lateral cervical mass simulating a branchial cleft anomaly was the sole presenting sign of the disease. These masses were nodal metastases of occult papillary adenocarcinoma of the thyroid that underwent liquefaction necrosis. Sonographically, the masses presented a complex pattern. Accordingly, it is suggested that, in patients presenting with an asymptomatic solitary lateral cystic cervical mass, the possibility of metastasis from an occult thyroid malignancy should be considered, and consent for definitive surgery should be obtained. The ultrasonic pattern of the cystic mass is of importance in the differentiation of a cavitated lymph node from a branchial cleft cyst.     

Surgical management of the cleft sternum

Cleft sternum is a rare disturbance of unknown origin in the normal central midline fusion. Three cases of upper cleft sternum are reported here, one associated with a pericardium to the skin sinus tract. Surgical correction of this anomaly in the neonatal period is advised, whether the infant is symptomatic or not. Simple closure of the defect during the first month of life avoids the more complex reconstruction necessary in older children.     

Unusual patterns of congenital neck masses in children

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)     

Branchial cleft cyst manifesting as hypoglossal nerve palsy

Hypoglossal nerve palsy occurred in 2 patients with infected second branchial arch cleft cyst. This very unusual complication of the congenital anomaly has been related to the mechanical compression of the mass. Histologic features of the perineural coat were also suggestive for this etio-pathogenesis.     

Branchial anomalies in the pediatric population.

OBJECTIVE: We sought to review the presentation, evaluation, and treatment of branchial anomalies in the pediatric population and to relate these findings to recurrences and complications. STUDY DESIGN AND SETTING: We conducted a retrospective study at a tertiary care pediatric hospital. PATIENTS: Ninety-seven pediatric patients who were treated for branchial anomalies over a 10-year period were reviewed. Patients were studied if they underwent surgical treatment for the branchial anomaly and had 1 year of postoperative follow-up; 67 children met criteria, and 74 anomalies were studied. RESULTS: Patients with cysts presented at a later age than did those with branchial anomaly fistulas or sinus branchial anomalies. 32% of branchial anomalies were previously infected. Of these, 71% had more than one preoperative infection. 18% of the BA were first arch derivatives, 69% were second arch derivatives and 7% were third arch derivatives. There were 22 branchial cysts, 31 branchial sinuses and 16 branchial fistulas. The preoperative and postoperative diagnoses differed in 17 cases. None of the excised specimens that contained a cystic lining recurred; all five recurrences had multiple preoperative infections. CONCLUSIONS: Recurrence rates are increased when there are multiple preoperative infections and when there is no epithelial lining identified in the specimen.