Excision of Branchial Cleft Cysts

EJ

FO

omiTeem

seftsct

ati(tcf

cpdtt

mC

1d

Operative Techniques in Otolaryngology (2005) 16, 213-222

xcision of branchial cystsohn Houck, MD, MS, FACS

rom the Department of Otorhinolaryngology, University of Oklahoma, Health Science Center, Oklahoma City,
klahoma.
Branchial cleft anomalies are uncommon findings that are often confused with other causes of massesand infections in the neck. Although more common in children, they may also present in adults. Secondcleft anomalies are 8-10 times more common than those of the first. Third and fourth cleft anomaliesare quite rare. All offending epithelium must be surgically removed. A thorough understanding of theembryologic origin, presentation, diagnostic evaluation, and surgical treatment of these challenginglesions will help the head and neck surgeon to a good outcome.© 2005 Elsevier Inc. All rights reserved.

KEYWORDSCongenital anomalies;Branchial apparatus

petd

dvstlt

A

P

naeioe

timfsmblo

The branchial apparatus appears during the fourth weekf gestation, consisting of 4 well-defined paired sets ofesodermal arches, divided by endothelial-lined pouches

nternally and ectodermal lined clefts (grooves) externally.hey are sequentially numbered from cranial to caudal. Thectoderm of each cleft is initially in contact with thendoderm of its pouch, forming a double-layered branchialembrane (Figure 1).The mesenchyme of each arch initially comes from me-

oderm, which gives rise to skeletal muscle and vascularndothelium. During the fourth week, neural crest cellsrom the mid and hindbrain migrate into the arches, con-ributing to the mesenchyme that produces most of thekeletal and connective tissue structures. Each branchial archontains a cartilaginous structure (skeleton), muscle, a nervehat supplies the muscles and mucosa, and an artery (Figure 2).

During the fifth week, the ventral aspect of the secondrch enlarges and grows caudally to overlap the second,hird, and fourth arches. These arches coalesce and sinkn to form an ectodermal depression, the cervical sinusFigure 3). By the seventh week, the cervical sinus andhe second to fourth grooves disappear, giving a smoothontour to the neck. The only remaining groove, the first,orms the external auditory canal (EAC) (Figure 4).

The first branchial pouch elongates to form the eusta-hian tube, middle ear, and mastoid antrum. The secondouch develops into the tonsillar fossa. The third pouchevelops into a dorsal portion, which forms the inferiorhyroid gland, and a ventral portion that gives rise to thehymus. These primordial structures initially connect to the

Address reprint requests and correspondence: John Houck, Depart-ent of Otorhinolaryngology, University of Oklahoma, Health Scienceenter, PO Box 26901, WP 1360, Oklahoma City, OK 73190-3048.

fE-mail address: [email protected].

043-1810/$ -see front matter © 2005 Elsevier Inc. All rights reserved.oi:10.1016/j.otot.2005.09.007

harynx by the thymopharyngeal duct, which then degen-rates. The inferior parathyroid glands migrate caudally tohe dorsal surface of the thyroid gland, and the thymusescends into the superior mediastinum.

The fourth pouch develops similarly to the third, with aorsal portion developing into superior thyroid glands and aentral portion developing into the ultimobranchial body. Thistructure, which is formed by neural crest cells, fuses with thehyroid gland, giving rise to the calcitonin producing parafol-icular cells (C cells) (Figure 5). The only branchial membranehat persists is the first, which forms the tympanic membrane.

nomalies

athogenesis

The abnormal persistence of branchial apparatus rem-ants gives rise to branchial anomalies (ie, cysts, fistulae,nd sinuses). A cyst is lined by epithelium but has noxternal opening. A sinus is a blind pocket that opensnternally (persistence of a pouch) or externally (persistencef a cleft). A fistula is a tract that has both internal andxternal openings.

The origin of fistulae and sinuses seems directly relatedo the branchial apparatus as a result of the anatomic sim-larities. However, the origin of lateral cervical cysts re-ains controversial. A likely explanation is that cysts form

rom entrapped remnants of either pouches, clefts, or per-istent ectoderm of the cervical sinus. Histologically, squa-ous epithelium lines most cysts, although some are lined

y respiratory (pseudostratified ciliated columnar) epithe-ium and some by both types of epithelium. The cysts areften lined by subepithelial lymphoid tissue, replete with
ollicles and germinal centers. However, sinuses and fistulae

as

aadpg(v

iwefi

F

biml

bbmEtocal(hicop

fi

Fw

Fa

F

214 Operative Techniques in Otolaryngology, Vol 16, No 3, September 2005

re more likely to be lined by respiratory epithelium. As-ociated lymphoid tissue tends to lack well-formed follicles.

The thymopharyngeal duct theory suggests that the cystsre remnants of the original connection between the thymusnd third pouch. However, a persistent thymopharyngealuct has not been found, and the cysts only rarely havearathyroid or thymic tissue in them. Another theory sug-ests that cysts result from deposition of salivary glandparotid) inclusions within cervical lymph nodes, but sali-ary tissue is only rarely seen in the cysts.

Another theory suggests that epithelial metaplasia occursn cervical lymph nodes. Up to 96% of specimens containell-differentiated lymphoid tissue that cannot easily be

xplained by a branchial origin. Also, many of these cystsrst become apparent during a respiratory tract infection.

irst branchial cleft

Several classification schemes for these anomalies haveeen proposed, the most widely accepted being that of Workn 1972.1,2 Type I lesions are duplication anomalies of theembranous EAC, ectodermal in origin and without carti-

igure 1 Horizontal section through the cranial region at 3-4eeks.

age (Figure 6). Type II lesions are duplication anomalies of s

oth the membranous and cartilaginous EAC, containingoth skin and cartilage from both ectodermal and mesoder-al origins. Anatomically, type I lesions run parallel to theAC, involving parotid tissue and usually passing superior

o the facial nerve. They usually are parallel to the EAC andpen in the preauricular region. Type II lesions are moreommonly located within the substance of the parotid glandnd more closely related to the facial nerve, running eitherateral, medial, or even through the main trunk of the nerveFigure 7). With time, exceptions to the Work classificationave been reported, with cysts classified as type I, contain-ng both cartilage and skin, and cysts classified as type II,ontaining only skin.2 Newer classification schemes stressnly that a lesion be a cyst, fistula, or sinus for adequatereoperative planning.

Approximately 8% to 10% of all branchial anomalies arerst cleft origin. The lesions have been found in all age groups,

igure 2 Skeletal and ligamentous derivatives of the branchialrches.

igure 3 Early formation of the branchial arches and cervical
inus.

mpwseeniac

D

y

ca

T

mip

215Houck Excision of Branchial Cysts

ore often on the left side. There is probably no genderredominance. Cysts occur twice as often as sinuses or fistulae,ith cystic lesions more common in adults, and fistulae and

inuses more common in children. The location of cysts andxternal sinus openings varies, usually occurring around thear (anterior, inferior, and posterior to the pinna) or in the uppereck, anterior to the sternocleidomastoid muscle (SCM). Thenternal tracts for both sinuses and fistulae are most often foundlong any portion of the EAC, with the anteroinferior bony-artilaginous junction being most common.

iagnosisDiagnosis can be difficult, with an average delay of 4

ears, often only after infection. Preoperative plain films,

Figure 4 Horizontal section at 7 weeks.

Figure 5 The fourth pouch.

omputerized tomography, or magnetic resonance imagingre probably of little value.

reatmentTreatment is complete surgical removal of all abnor-

ally placed epithelium. Surgery should wait until acutenfection has resolved. Incision and drainage may be appro-riate for an abscess. Repeated infections and limited sur-

Figure 6 First branchial cleft cyst, type I.

Figure 7 First branchial cleft cyst, type II.

ga

feseitaeecfi

S

awaapsm

panicjc

C

pTtuSt

D

cdte

ial cle


eries (incision and drainage [I�D]) lead to more scarringnd a more difficult definitive procedure.

A standard cervicomastoid parotidectomy incision withormal dissection of the facial nerve and a lateral parotid-ctomy are recommended to give the best chance of pre-erving the facial nerve and removing all the offendingpithelium. Injection of the tract with methylene blue ornsertion of a wire probe may assist with identification ofhe tract. If the tract extends to the EAC, adjacent canal skinnd cartilage should be removed. Rarely, the tract mayxtend to the middle ear space or even extend along theustachian tube. Transient weakness of the facial nerve isommon. Recurrence is unlikely but is more common withbrosis from prior infections and surgery.

econd cleft and pouch

These defects represent 67% to 93% of all branchialnomalies; approximately 10% are bilateral.3 Such lesionsould be expected to pass deep to second arch structures

nd over third arch structures. Thus, a fistula extends fromn external opening along the anterior border of the SCM,ierces the platysma muscle, and then follows the carotidheath upward to the level of the hyoid bone. It then turns

Figure 8 Second branch

edially to pass over the hypoglossal nerve, beneath the b

osterior belly of the digastric muscle, between the internalnd external carotid arteries, over the glossopharyngealerve, deep to the stylohyoid ligament to an internal open-ng at the upper pole of the tonsillar fossa. Although cystsan occur anywhere along this tract, they are most commonust lateral to the internal jugular vein, at the level of thearotid bifurcation (Figure 8).

ystsCysts occur 3 times more often than fistulae and tend to

resent in an older group, the second to fourth decades.here is probably no gender or side predominance. Presen-

ation is typically a painless, smooth, round neck mass,sually along the upper third of the anterior border of theCM, appearing often after an upper respiratory tract infec-

ion. Acute infection can lead to abscess.

iagnosisThe correct diagnosis is made in approximately half the

ases. Preoperative computerized tomography or fine-nee-le aspiration helps to distinguish cysts from carotid bodyumors, cystic metastases of squamous cell carcinoma, ad-nopathy, lymphoma, cystic hygroma, neurofibromas, tu-

ft cyst. CN, cranial nerve.

erculosis, and parotid gland lesions.

T

msAdIss

smpf1sde

S

ccvcaTu

crpu

gmIjtm(

fnowtcwr

C

saa

S

nthmft

r secon


reatmentComplete surgical removal of all offending epithelium

ust be performed, preserving the surrounding blood ves-els and nerves, using a cosmetically acceptable incision.cute infections must first be treated, including incision andrainage for abscesses. Similar to first cleft cysts, repeated�Ds or insufficient surgery makes complete surgical exci-ion more difficult, with damage to critical neurovasculartructures and recurrence more likely.

Because most of these patients are younger, cosmesis ofurgical access is an important issue. Incisions should beade along Langer lines, generally directly over the mid-

oint of the cyst (Figure 9). Additional exposure may beacilitated through a McFee, or stair step, incision (Figure0). Vertical incisions should be avoided because of un-ightly scars. Partial aspiration of some cysts may helpissection. Cysts lying medial to the carotid sheath are moreasily removed transorally.

urgical procedureInferior and superior subplatysmal flaps are raised. The

yst is separated from the superficial layer of the deepervical fascia that envelopes the SCM. Superficial jugulareins and cutaneous nerves are transected (Figure 11). Theyst is then dissected off the SCM, preserving the spinalccessory nerve. Dissection is easier if the cyst is intact.raction with forceps must be gentle to avoid rupture (Fig-re 12).

The cyst is dissected medially, carefully avoiding thearotid sheath and ansa hypoglossi. The facial vein mayequire transaction (Figure 13). The cyst is dissected off theosterior belly of the digastric and stylohyoid muscles (Fig-

Figure 9 Incision fo

re 14). Posteriorly, the cyst or its tract crosses the hypo- m

lossal nerve (XII) and the veins overlying it. The veinsust be dissected or transected carefully to avoid bleeding.

ndiscriminate clamping because of bleeding can easily in-ure XII. The tract crosses the glossopharyngeal nerve nearhe pharyngeal attachment. The superior laryngeal nerve isuch deeper, passing obliquely behind the external carotid

Figure 15).The duct of the cyst can be difficult to differentiate from

ascial attachments. If present, it passes between the exter-al and internal carotid arteries, deep to the posterior bellyf the digastric and stylohyoid muscles (Figure 16). Theound is irrigated and closed in multiple layers, including

he superficial layer of the deep cervical fascia. A carefulosmetic closure is performed, such as a subcuticular sutureith Steri-Strips (3M, St. Paul, MN), for a good cosmetic

esult in a young person.

omplicationsThe most common complication is recurrence. One large

eries of 208 cases showed recurrence in 21% of those withhistory of prior surgery, 14% with a history of infection,

nd 3% with a history of neither.

inuses and fistulaeMost are noted shortly after birth, with 78% being diag-

osed before age 5 years. The opening onto the neck isypically along the anterior border of the SCM, between theyoid bone and suprasternal notch. It may drain mucoidaterial, which may become purulent during an acute in-

ection. They occur slightly more often in females and onhe right side. Fistulae are less common than sinuses. They

d branchial cleft cyst.

ay be lined with squamous or respiratory epithelium.

Lp

D

ot

T

l

bMs

ocdlDDy

stair


ymphoid tissue tends to be scanty and not have a follicularattern like that in cysts.

iagnosisPreoperative imaging is not likely to be helpful. Injection

f the tract with contrast material may show the extent ofhe lesion and assist with preoperative planning.

reatmentComplete surgical excision is necessary and can be de-

ayed until an infant is 2-3 years old. Acute infection should

Figure 10 A

Figure 11 Initial ex

e treated and resolved before surgery. A “stepladder” orcFee incision will gain adequate access and leave less

carring than a vertical incision.The tract opening is excised along with an elliptical piece

f surrounding skin. Dissection proceeds through the sub-utaneous tissues, platysma, and the superficial layer of theeep cervical fascia. The second incision is made about theevel of the hyoid bone and the tract threaded through it.issection can easily proceed to remove the entire tract.issection may be assisted by injecting the tract with meth-lene blue or inserting a flexible catheter, such as an em-

step incision.

posure of cyst.

bee

C

R

T

abd

ss

sfssgp1

trs

tion of


olectomy catheter. Enough supporting tissue should bexcised with the tract to ensure complete removal of allpithelium.

omplicationsDysfunction of the hypoglossal nerve is usually transient.

ecurrences may require functional neck dissection.

hird and fourth pouch

Anomalies are relatively uncommon, with third archnomalies at 2% to 8% and fourth at 1% to 2% of allranchial anomalies.4,5 Distinguishing between the 2 can beifficult because both enter the pyriform sinus and have

Figure 12 Further dissec

Figure 13 Media

imilar presentations. Tracts originating in the pyriforminus have been associated with recurrent acute thyroiditis.

Theoretically, a third arch pouch and cleft anomalyhould pass between third and fourth arch structures. There-ore, a complete fistula would then start in the pyriforminus, pierce the thyroid-hyoid membrane, pass above theuperior laryngeal and hypoglossal nerves, go beneath thelossopharyngeal nerve and carotid artery, pierce thelatysma and surface in the skin anterior to the SCM (Figure7).

Theoretically, a fourth pouch anomaly would pass be-ween the fourth and sixth arch structures (the fifth arch isudimentary). A complete fistula would start in the pyriforminus, exit the larynx near the cricothyroid joint, pass be-

cyst with careful traction.

l dissection.

tdwtTttpn

bpHat

hcssrc

D

avhpv

issecti


ween the superior and recurrent laryngeal nerves, then trackown the tracheoesophageal groove. On the left side, itould curve around the aorta from posterior to anterior. On

he right side, it would curve around the subclavian artery.he tract would then lie posterior to the carotid, pass over

he hypoglossal nerve, and exit near the anterior border ofhe SCM. A fourth arch fistula has never been described; aossible fourth pouch sinus tract has been described but wasot completely dissected inferior to the clavicle.6

Clinically, both types begin at the pyriform sinus and endlindly in the paratracheal or thyroid regions. The fourthouch sinuses only rarely extend beneath the clavicles.istologically, both third and fourth pouch tracts may be

ssociated with ectopic thymus and aberrant parathyroidissue (both third pouch).7

Figure 14 D

Figure 15 The hyp

Most fourth pouch anomalies are diagnosed in child-ood, and 97% occur on the left side. There are 2 types oflinical presentation. In the first, lateral neck cysts or ab-cesses occur in neonates and may be a cause of neonataltridor.7 In the second, recurrent lateral neck abscesses orecurrent episodes of acute suppurative thyroiditis present inhildren, adolescents, and occasionally adults.8

iagnosisThe diagnosis can be quite difficult, often being made

fter repeat admissions for incision and drainage, and intra-enous antibiotics. A plain film showing air in the cyst canelp differentiate a branchial anomaly from other causes ofediatric neck masses. The pharyngeal opening may beisualized by flexible fiberoptic nasopharyngoscopy. A bar-

on superiorly.

oglossal nerve.

imtTpcmp

T

odamc

tblnfsp

sanbjiltdpnir

Figure 16 Dissectio

Figure 17 Third arch anomaly. CN, cranial nerve.


um pharyngoesophagram will show the sinus tract in al-ost every case, if 6 weeks are allowed to pass after infec-

ion to let associated inflammation and edema to subside.9,10

he initial episode of acute thyroiditis in a child shouldromote a search for a pharyngeal sinus. Direct laryngos-opy should be performed before any surgical excisions anday be most easily performed at an incision and drainage

rocedure.

reatmentThe tract must be completely excised, including the area

f the pyriform sinus attachment. The procedure can beifficult and should be performed only in the absence ofcute infection. Some have found injection of the tract withethylene blue or catheterization with an embolectomy

atheter helpful.The tract must be removed surgically. The relationship of

he tract to the recurrent and superior laryngeal nerves muste determined. If the tract goes inferior to the superioraryngeal nerve (fourth arch) and superior to the recurrenterve (sixth arch), then the anomaly is derived from theourth pouch. However, if the tract passes superior to theuperior laryngeal nerve and inferior constrictor, then a thirdouch origin is likely.

To permit visualization and resection of the pyriforminus origin, the thyroid ala should be retracted medially,nd the inferior constrictor muscle should be divided. Ifecessary, a vertical strip of posterior thyroid cartilage cane excised after disarticulation of the ipsilateral cricothyroidoint. After removing the pharyngeal attachment, the defects closed with a purse-string suture.7 Partial or total thyroidobectomy may be needed for fourth pouch sinuses. Dissec-ion of the recurrent laryngeal nerve may be needed toistinguish clearly this nerve from the tract. The superiorarathyroid gland should be preserved. Fourth pouch rem-ants terminate in the prethyroid space or within the glandtself. Care must be taken to preserve the superior parathy-

n of the duct.

oid gland.

C

y

R

1


omplicationsComplications include recurrent nerve vocal cord paral-

sis or pharyngocutaneous fistula.

eferences

1. Work WP, Proctor CA: The otologist and first branchial cleft anom-alies. Ann Otol Rhinol Laryngol 72:548-562, 1963

2. Work WP: Newer concepts of first branchial cleft defects. Laryngo-scope 82:1581-1593, 1972

3. Gatti WM, Zimm J: Bilateral branchial cleft fistulas: Diagnosis and
management of two cases. Ear Nose Throat J 67:256, 258, 261, 1988
4. Choi SS, Zalzal GH: Branchial anomalies: A review of 52 cases.Laryngoscope 105:909-913, 1995

5. Ford GR, Baladrishnan A, Evans JNG, et al: Branchial cleft and pouchanomalies. J Laryngol Otol 106:137-143, 1992

6. Shugar MA, Healy GB: The fourth branchial cleft anomaly. HeadNeck Surg 3:72-75, 1980

7. Rosenfeld RM, Biller HF: Fourth branchial pouch sinus: Diagnosisand treatment. Otolaryngol Head Neck Surg 105:44-50, 1991

8. Narcy P, Aumont-Grosskopf C, Bobin S, et al: Fistulae of the fourthendobranchial pouch. Int J Pediatr Otorhinolaryngol 16:157-165, 1988

9. Lin JN, Wang KL: Persistent third branchial apparatus. J Pediatr Surg26:663-665, 1991

0. Godin MS, Kearns DB, Pransky SM, et al: Fourth branchial pouchsinus: Principles of diagnosis and management. Laryngoscope 100:
174-178, 1990

Documents

Excision of Branchial Cleft Cysts