Congenital Neck Masses - Department of Surgery at SUNY...

Congenital Neck Masses

C. Stefan Kénel-Pierre, MD

SUNY-LICH Medical Center

Department of Surgery

www.downstatesurgery.org

Case Presentation

• xx year old male presents with sudden onset left lower neck swelling x 1 week

• Denies pain, shortness of breath, dysphagia

• No history of trauma, recent upper respiratory infection

• NKDA

• Medications: none


Physical Exam

• Large swelling in lower half of left neck encompassing lower and medial portions of sternocleidomastoid muscle

• Does not move with deglutition

• No visible sinuses, erythema, bruit appreciated on exam

• No cervical/supraclavicular/axillary lymphadenopathy


Laboratory Values

142 105 19 92

3.8 28 1.0 7.0

13.5

189

41.1


Studies

• U/S guided needle aspiration, pathology negative for malignant cells; demonstrated keratinized squamous cell epithelial cells

• CT Neck: large left lower neck cystic mass measuring 3.5x4.2x7.2 cm, posterior to left sternocleidomastoid muscle. Mass compressing left IJ. Carotid arteries, larynx and periglottic spaces are unremarkable.


Operative Findings

• Massive, deep-seated 6x7cm branchial cleft cyst wrapped around lower 2/3 of carotid sheath

• Vagus, hypoglossal nerves were intimately associated with mass, sharply dissected and preserved

• Estimated blood loss was negligible


Post Operative Recovery

• Patient tolerated procedure well

• Immediate postoperative course complicated by urinary retention

• Discharged home postoperative day 1

• Postop office visit, pt fully recovered

• Pathology consistent with cervical lymphoepithelial cyst (second branchial cyst)


Presenter

Presentation Notes

Cyst wall 0.4 cm thick. Measures 3.5x2.5x2.5cm in size


Presenter

Presentation Notes

Before I begin our discussion on congenital lesions, I think it best to go over the embryology associated with neck masses

Branchial Apparatus

• Consists of arches, pouches and clefts

• Arches develop from buds in lateral pharynx

• Ectoderm lined clefts separate arch externally

• Endoderm lined pouches separate internally

• Maldevelopment may result in a neck mass.


Presenter

Presentation Notes

The branchial apparatus consists of arches, pouches and grooves or clefts. The branchial arches develop from mesodermal buds in the lateral walls of the fetal pharynx. Each arch contains a core cartilaginous skeleton, a cranial nerve, an artery and rudimentary muscle.


Presenter

Presentation Notes

For those of you who, like myself are more visually oriented, perhaps this graphic will better allow you to picture the branchial apparatus

Branchial Arch Derivatives

• First Branchial arch (mandibular) – Meckel’s cartilage (forms malleus/incus)

– Muscles of mastication

– Supplied by trigeminal nerve and external maxillary artery

– Pouch forms eustachian tube, mesotympanum & mastoid antrum



• Second Branchial Arch

– Reichert’s cartilage (hyoid bone, styloid process)

– Muscles of facial expression

– Supplied by facial nerve and stapedial artery

– Pouch forms mesotympanum and palatine tonsil



• Third Branchial Arch

– Greater cornu and body of hyoid bone

– Stylopharyngeus, constrictor muscles of pharynx

– Supplied by glossopharyngeal nerve and common

carotid artery

– Pouch forms thymus and inferior parathyroids.



• Fourth Branchial Arch

– Thyroid cartilage

– Cricothyroid, inferior pharyngeal constrictor

– Supplied by superior laryngeal nerve and aortic

arch, subclavian artery

– Pouch forms superior parathyroid glands



• Fifth/Sixth Branchial Arches

– Cricoid/Arytenoid cartilages

– Intrinsic laryngeal

– Supplied by recurrent laryngeal nerve and ductus

arteriosus/pulmonary artery

– Clinically silent


Congenital Neck Masses

• Branchial Cleft Cyst

• Thyroglossal duct cyst

• Cystic Hygroma

• Dermoid Cyst

• Thymic Cyst

• Ranula


Branchial Cleft Anomalies

• Majority originate from 2nd pharyngeal cleft

• Failure of obliteration of cervical sinus of His

• Present as cysts, sinuses or fistulae

• Lined with squamous epithelium

• Often present with recurrent infection in 2-4th decade of life


Presenter

Presentation Notes

Branchial cleft anomalies can originate from any arch, however most frequently they originate from the second branchial cleft. About 1-8% however will originate from the first branchial cleft and present as a swelling near the tragus, postauricular sulcus (type I) or below the angle of the mandible (type II)

Second Branchial Cleft Anomalies

• Sinuses/fistulae occur in lower third of the neck and may be bilateral in 1/3 of cases

• Intimately associated with neurovascular bundle, unlike thyroglossal cyst

• Recurrence is rare, unless infected

• Cysts manifest deep to sternocleidomastoid, near carotid bifurcation/parapharyngeal space



Presenter

Presentation Notes

As you can see from this axial cut, the branchial cleft cyst lies at the bifurcation of the carotid artery, just deep to the sternocleidomastoid

Branchial Cleft Sinuses/Fistulae

• Sinuses: openings on the skin or tonsillar fossa

• Fistulae: communication between skin and pharynx

• Less common than cysts

• Present within first decade of life

• Are bilateral in 20% of cases

• Slight female predominance


Thyroglossal Duct Cyst

• Appear as a painless midline mass

• Present at birth, may not become apparent until adulthood

• Excision indicated, risk of infection/malignancy

• Thyroglossal duct carcinoma arises within thyroid tissue often accompanying epithelial cystic remnants


Presenter

Presentation Notes

Typically found overlying the hyoid but can be found anywhere along the course of the thyroglossal duct. Malignancy is observed in less than 1%. Often present as anterior midline neck masses in the 1st decade of life. More than 25% present before age 5 and 40% present by age 10. 10% per decade thereafter.

Diagnosis

• Diagnosis is based almost entirely on history and physical

• Elevation and movement of lesion with deglutition and protusion of tongue is pathognomonic

• Intraoperatively appear thin walled and translucent


Presenter

Presentation Notes

Presence of solid mass should prompt identification of thyroid prior to excision of partially descended gland. A nontranslucent cyst should be aspirated, to identify dermoid cyst, as excision would not include hyoid)

Surgical Treatment

• Sistrunk Procedure involves complete excision of mass, including central portion of hyoid

• Prior to excision, need to confirm presence of normal thyroid tissue.

• Stalk dissected to level of foramen cecum and suture ligated

• Care should be taken not to rupture cyst


Presenter

Presentation Notes

Risk of recurrence is significantly reduced from 25 to less than 5% when compared with cystectomy alone, most within first year postoperatively. Dissection of the tract can be aided by digital pressure on the base of tongue by anesthesiologist.

Dermoid Cysts

• Consists of tissue for all three germinal layers

• Typically present as midline neck masses

• Rarely present while acutely infected

• Do not move with swallowing

• U/S may not be able to distinguish between dermoid cysts and thyroglossal duct cysts

• Treatment is cystectomy alone


Presenter

Presentation Notes

May contain skin appendages, sweat glands/sebaceous glands. Important to correctly identify cysts prior to excision of hyoid, as this is superfluous in dermoid cysts.

Cystic Hygroma

• Lymphatic malformations from failure of drainage into venous system

• Represent less than 5% of congenital neck masses

• Tend to develop in left posterior triangle

• Present in the first two years of life

• Associated with spontaneous hemorrhage


Presenter

Presentation Notes

Can occur anywhere, most (>75%) involve the lymphatic jugular sac in the posterior neck, 20% in axilla. Cysterna chili and formal horacic duc develop by 10th week

Clinical Presentation

• Soft, compressible, cystic masses that distort

surrounding structures

• Multiloculated cystic structures on u/s or CT

• Vascular malformations may be identified

within cystic hygroma


Surgical Resection

• Intimate anatomic involvement may impede complete excision

• Do not follow native tissue planes

• Drainage often utilized to prevent seroma formation in resection of moderate to large lesions

• Variable recurrence rate (6-50%)


Presenter

Presentation Notes

Other therapeutic options include cryotherapy, diathermy and sclerotherapy (picibanil) exacerbate infection and marginally successful. Watch for airway compromise from inflammatory response.

Thymic Cyst

• Occur anywhere in lower neck, mostly left side

• Present during the first decade of life

• Male predominance, nearly twice as frequent

• Firm, compressible round lesions, confused with branchial cleft anomalies


Presenter

Presentation Notes

Can occur anywhere in the lower neck, mostly on the left side from the piriform sinus to the chest

Ranula

• Post inflammatory retention cyst of sublingual gland

• Usually involve major salivary glands

• Can fluctuate rapidly in size

• Treatment involves either excision or marsupialization


Presenter

Presentation Notes

Although not a congenital cyst, a ranula, particularly the variant known as diving ranula, can be confused with manyRana is from the Latin… means frog

Summary

• Congenital masses arise from maldevelopment of

branchial apparatus or thyroid

• Presentation can vary based on location

• Knowledge of anatomy and embryologic variants

is vital to surgical technique

• Imaging studies aid in determining resectability


References

• Mastery of Surgery, 5th Edition, Fischer et al

• Pediatric Otolaryngology, The Requisites,

Wetmore et al.

• Otolaryngology, 5th Edition, Cummings et al.

• Textbook of Surgery, 19th Edition, Sabiston et

al.


Congenital Neck Masses - Department of Surgery at SUNY...

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