Volume 2, Issue 4 , Pages 222-224, December 2007
Oropharyngeal second branchial cleft cyst
Article Outline
Summary
Second arch branchial cleft cysts although a frequently occurring branchial anomaly is almost always located in neck. Its presence in oropharynx is extremely rare. We report a rare case of second branchial cleft cyst measuring 1
cm×1.1cm in oropharynx which presented with nasal obstruction and difficulty in swallowing. This cyst was excised and biopsy revealed squamous lined epithelial wall with lymphoid aggregation which is characteristic finding of branchial cleft cyst. Six months follow up of the child was uneventful and there was no recurrence. A review of theories for genesis of branchial cleft cysts is also done.
Keywords: Second arch branchial cleft cysts, Branchial cysts, Neck mass, Tonsillar mass, Oropharyngeal mass, Congenital neck mass
1. Introduction
Most (95%) branchial cleft anomalies arise from the second cleft [1]. Second branchial clefts originate from remnants of cervical sinus of His and its duct during differentiation of branchial apparatus. Usually the second branchial pouch gives rise to the palatine fossa and tonsils. The classic location of a branchial cyst is anterior and deep to the sternocleidomastoid muscle at the level of the carotid bifurcation [2]. It can occur anywhere along the line from the tonsillar fossa to the supraclavicular area of the neck [1]. We present a rare case of second branchial cleft cyst with duct located at the superior tonsillar fossa extending into nasopharynx and also review its embryological basis.
2. Case report
An 18-month-old boy presented with complaints of difficulty in feeding and nasal obstruction. Parents of the boy had noticed a swelling in oropharynx for which they brought the child to OPD services of our hospital. Clinical examination revealed a whitish mass measuring 1cm×1.1cm in left superior tonsillar fossa encroaching soft palate and pushing the uvula to the other side. Oropharyngeal inlet was compromised due to the mass. Nasal endoscopy showed that the mass was not attached to the roof of nasopharynx and was obstructing the left posterior choana. Both fossa of rosenmuller, Eustachian tube orifice and examination of ears were normal. CT scan confirmed that the mass was solitary and not attached to any duct or skull base. MRI showed a well-defined lesion hanging from the uvula and soft palate in the midline showing hyperintense signal on T2 weighted and STIR (short tau inversion recovery) images and isointense signal on T1 weighted images with out any intracranial extension (Fig. 1). The cyst was excised transorally under general aneasthesia. It was found to be adhesive to the left superior tonsillar pillar. Histopathological examination of the cyst showed, on gross examination, a smooth walled cyst which was grey white in color. Microscopic examination revealed ciliated tall columnar respiratory epithelium lining the cyst wall. Due to its anatomical location and morphological similarity to branchial cyst, this cyst was reported as an intraoral branchial cleft cyst possibly arising from remnant of second branchial cleft (Fig. 2). Patient was discharged without any complication and there was no evidence of recurrence after 6 months of follow up.
Fig. 1.
MRI axial and coronal sections.
Fig. 2.
Histopathological picture of branchial cyst at 100×.
3. Discussion
Branchial cleft anomalies may manifest as sinus, fistula, cyst or skin tags, and cartilage. Any combination of these anomalies may occur. An isolated cyst may be present which may have no connection to the skin or pharynx. Although cystic anomalies are more common in adolescents and adults, in our case in which oropharyngeal cyst was present, child was symptomatic much earlier in life. Three theories have been postulated to describe the etiology of internal branchial cysts. The first suggests that, due to the presence of sub epithelial lymphocytes, the cyst is most likely derived from ectopic epithelial cells in the regional lymph node [3]. The second theory, which is more widely accepted, suggests that the cysts are derived from remnants of the branchial apparatus. The third theory is that cysts originate from epithelial cells desquamated during repeated infections of the pharyngeal mucosa and reach regional lymph nodes through lymphatic vessels [3]. The outgrowth and subsequent degeneration of these cells may then form the cystic lesions of the neck. Histologically these cysts are circumscribed cysts, 2–5cm in diameter, with fibrous walls usually lined by stratified squamous or pseudostratified columnar epithelium underlain by an intense lymphocytic infiltrate or, more often, well-developed lymphoid tissue with reactive follicles. The cystic contents may be clear, watery to mucinous fluid or may contain desquamated, granular cellular debris [4].
Bailey has divided second branchial cleft cysts have been divided into four subtypes [5]. Type 1 cysts are located superficially along the anterior border of the sternocleidomastoid muscle beneath the cervical fascia. Type 2 cysts lie on the great vessels beneath the enveloping fascia of the neck, while type 3 pass between the great neck vessels to reach the pharyngeal wall. Type 4 cysts are situated under the pharyngeal wall medial to the great neck vessels. Our case had type 4 cyst which is thought to be extremely rare [5]. MRI is the investigation of the choice as it clearly differentiates the cyst from cystic hygroma, hemangioma, mucus retention cyst, lipoma, lymphoma and rhabdmyosarcoma. The classical findings are well-defined cyst wall without contrast enhancement (unless infected) and hypointense T1 images and hyperintense T2 weighted images. High protein content may cause the T1 images to be isointense or hyperintense as in our case [6]. Due to progressive air way compromise with dysphagia surgery was performed. Most authors have favored per oral removal of such pharyngeal branchial cysts [7]. However in cases of recurrence or incomplete removal of cyst due to adhesions, some authors have suggested lateral neck approach. We encountered minor adhesions during the surgery but it was managed. We conclude that for successful management of pediatric oropharyngeal masses, possibility of branchial cleft cyst should be kept in mind and MRI study should be done for all such cases.
References
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PII: S1871-4048(07)00053-6
doi:10.1016/j.pedex.2007.06.005
© 2007 Elsevier Ireland Ltd. All rights reserved.
Volume 2, Issue 4 , Pages 222-224, December 2007
