Head and Neck Sinuses and Masses Flashcards
An infant presents with a draining sinus on the anterior border of the sternocleidomastoid. What is the most likely diagnosis?
A. Second brachial cleft anomaly B. Third brachial cleft anomaly C. Thyroglossal duct cyst D. Cystic hygroma E. Dermoid cyst
ANSWER: A
COMMENTS: Embryologic development of the head and neck arises from the brachial arches.
Failure of complete closure results in fistulas, cysts, and sinuses.
The most common brachial cleft anomaly is from the second brachial cleft and generally presents in infancy with a draining sinus anterior to the sternocleidomastoid. In adolescence, this same anomaly will more commonly present as a cyst.
Once diagnosed, these should be resected since there is a risk of infection, enlargement, and malignant transformation.
These abnormalities often present as infected sinuses or cysts, and management is initial incision and drainage with definitive resection later.
Remnants from the third brachial pouch are very rare.
Thyroglossal duct cysts present as a midline mass that moves superiorly with tongue protrusion.
Cystic hygromas are congenital lymphangiomatous malformations that present in the posterior neck.
Dermoid cysts of the head and neck arise from elements trapped during fusion of the brachial arches.
They are usually found in the midline, are well-circumscribed, and are filled with sebaceous material that can become infected.
A 2-year-old boy is seen in your office with a midline neck mass that has been present for 2 months. On examination, the mass is 2 cm in size, is not tender or pulsatile, and moves with the protrusion of his tongue. Ultrasound of the neck demonstrates a midline cystic lesion sitting deep to the strap muscles with no surrounding lymphadenopathy or other pathology. The thyroid gland is noted in the normal location. Findings on thyroid function studies are normal. Which of the following statements is true regarding this mass?
A. Simple excision of the mass is sufficient.
B. This mass most likely represents ectopic thyroid tissue.
C. The rate of recurrence is very high after an appropriate therapy.
D. These lesions can be found along the base of the tongue and hyoid bone.
E. Most lesions are associated with a draining cutaneous fistula tract.
ANSWER:
D
COMMENTS: The most likely diagnosis is a thyroglossal duct cyst.
The differential diagnosis for a midline neck mass in a child would also include a dermoid cyst, lymphadenopathy, ectopic thyroid, thymic cyst, or a ranula (a mucocele of a salivary gland).
Thyroglossal duct cysts arise from remnants of the thyroid gland that descended from the foramen cecum at the tongue base down to its anatomic position in the neck.
The hyoid bone contains the tract from the foramen cecum, and the cyst is superficial to this.
Most do not have a draining fistula tract, as is common with branchial cleft remnants.
If a normal thyroid is not palpated, an ultrasound and thyroid function tests should be performed prior to surgical resection.
Surgical treatment involves removal of the cyst tract along with the central hyoid bone—the Sistrunk procedure.
Simple cyst excision alone results in high rates of recurrence, whereas a Sistrunk procedure has reported recurrence rates of less than 5%.
What congenital head and neck masses are most common in infants and children?
Dermoid cyst, benign lymph node, thyroglossal duct cyst (TGDC), cysts associated with 1st, 2nd or 3rd branchial cleft cysts (BCC) are the most common. Often they do not present at birth but later in childhood, especially after a viral URI (TGDC or BCC).
Once present the mass may be persistent.
Unless actively infected, rarely acute increase in size, or have any signs such as overlying skin erythema and/or fluctuance.
Rarely will these cause airway compromise, dysphagia, or pain.
What non-congenital neck masses are most common in infants and children?
Torticollis (hematoma in sternocleidomastoid muscle) can occur in newborns as a classic firm “mass” in lateral neck noticeable shortly after birth, especially difficult vaginal birth of large size infant, forcep or vacuum delivery.
Non-congenital masses are commonly secondary to infectious etiology and reactive.
As lymph nodes enlarge there is central necrosis of the hilum due to inadequate blood supply to the entire node.
Reactive lymphadenitis also occurs with acute streptococcal pharyngitis/tonsillitis or mononucleosis.
Primary infectious lymphadenitis presenting on face or neck is rare but differential diagnosis should include non-tuberculous mycobacterial (NTM) lymphadenitis, bartonella or “Cat-Scratch”, especially if there is positive history of recent cat scratch.
Malignant masses are rare by always possible, including lymphoma, soft tissue sarcomas, and others.
How should location of mass influence one’s differential diagnosis?
Midline versus lateral neck should dictate differential diagnosis.
Midline neck mass is typically either a dermoid or TGDC, while lateral neck masses, especially anterior or medial to anterior border of sternocleidomastoid (SCM) muscle are likely BCC.
Reactive lymph nodes are rarely midline but if midline it’s most commonly a submental node above the hyoid cartilage.
Posterior or levels 2, 3, and 4 of neck are likely reactive lymph nodes, and supraclavicular is rare and can be lipoma if soft, vascular malformation, or rare malignant masses.
Often what feels like a larger node is a group of smaller accessory chain benign nodes.
What is the typical presentation of pediatric neck masses?
Most are asymptomatic and noticed by parents incidentally. Acute lymphadenitis of lateral, posterior neck, or retropharyngeal space are not painful, but can decrease neck range of motion.
Lymphadenitis is very firm and are rarely fluctuant until the absolute late stages of abscess.
Do NOT decide on whether to con- sult otolaryngology or consider need for incision and drainage of based on “feel” or presence of fluctuance.
Decision for surgical intervention and timing should be based on clinical severity (fever, elevated WBC, ultrasound confirmation of central necrosis and phlegmon evolving to necrosis, etc.) and not based on “rim enhancement” on CT imaging with contrast as the “gold standard”.
Phlegmon versus actual abscess are simply spectrum of the process.
Unless lymphadenitis is small and ultrasound confirms no drainable “fluid” collection, earlier surgical I&D provides early resolution of fever, minimize duration of hospitalization and need for systemic antibiotics.
What specific questions should be included when obtaining history for pediatric neck masses?
Ask if masses presented during concomitant acute illness, duration of mass, single or multiple, overlying skin discoloration, pain, size change, then always ask about exposure history such as exotic travel, exposure to tuberculosis, and recent cat scratches.
For those who live in rural areas or farm, ask if they use well water (risk for NTM).
Actinomycosis is a rare cause of neck masses which can mimic rhabodomyosarcoma, more common in the Midwest.
Ask for history of purulent drainage through skin indicating now a fistula, any recurrence of mass if intermittent, prior I&D by ED or others, prior surgery, his- tory of MRSA, constitutional symptoms like night sweats, weight loss, and family history (especially preauricular pits, fistulas, BCC and BC fistulas).
Which are the predominant sites of NTM involvement?
Mandibular border, submandibular area, and preauricular area.
What are the characteristic pathologic skin changes of NTM?
NTM has a characteristic “bluish” or “dusky” pink hue of the skin over the lymphadenitis, and often the lymph node is immediately subcutaneous, so skin is “stuck” to the infectious tissue.
What does histologic sectioning of NTM lesions show? [1]
Pseudoepitheliomatous epidermal hyperplasia, intraepithelial abscesses, transepidermal elimination and dermal granulomatous inflammation accompanied by necrosis and suppuration are likely seen on histopathology of immunocompetent patients.
For immunocompromised patients, there may be suppurative inflammation.
What to do during actual NTM cervical lymphadenitis “excision”?
Mark out an elliptical area carefully including involved discolored skin.
Injecting local may introduce acid fast bacilli (AFB) into deeper tissue.
Plan incisions to allow primary closure with minimal tension. Send tissue from the very periphery of lesion for AFB culture, not the central necrotic material.
After gentle dissection to create skin flaps, debride granulomatous unhealthy tissue manually using moist raytec and rub until health tissue remains.
Use 4-0 Polyglactin in interrupted fashion to close the subcutaneous layer, then 5-0 Poliglecaprone in running subcuticular fashion to close the skin without tension.
No drains needed.
Is there a difference between incisional versus excisional biopsy and outcomes? [2]
Complete excision is best whenever possible to reduce persistence or recurrence.
If not possible still remove as much as possible without risking marginal mandibular branch of facial (if submandibular location).
Medical treatment of NTM cervical lymphadenitis pre, intraop, and post op?
May treat pre- and post-operatively based on infectious disease consultation, but typically macrolide (azithro- or clarithromycin), add rifampin and even ethambutol for disseminated or systemic infections specifically pulmonary NTM.
For face and neck NTM, macrolide is sufficient if there is residual disease after excision.
What are complications of Sistrunk procedure or TGDC excision? [3, 4]
In a NSQIP review of adult cases from 2005 to 2014, 48/793 (6.1%) underwent reoperation.
Wound infection rates were higher in revision cases compared with primary operations (8.3% and 0.9%)
In a review of 99 pediatric TGDC excision cases from 2005 to 2015 from two university hospitals, with mean age of 4.4 years, seven were referred for recurrent TGDC.
Overall complication found in 24/99 (26.4%).
Hemorrhage and resection of thyroid cartilage were the most severe complications. Recurrence and wound infection (both n = 7, 7.7%) were most common.
Most devastating complication ENT sees that are not reported in literature, are rare cases performed by pediatric or general surgeons when there has been removal of the central portion of the cricoid instead of the hyoid cartilage.
This will result in potential life threatening subcutaneous emphysema and airway distress, and remedied by laryngotracheal reconstruction and repair of defect using costochondral rib cartilage.
Make a horizontal incision along natural neck skin crease line that is wide enough (usually medial to anterior SCM border from side to side) and across apex of TGDC or slightly above.
Elevate skin flaps and platysma, identify midline raphe, and lateralize strap muscles.
Expose and identify thyroid notch in order to dissect superiorly and find hyoid cartilage.
If you do not see the thyroid cartilage you may be too low. Dissect TGDC first then follow superiorly all the way to the hyoid.
The cricoid is the hardest tracheal ring on palpation.
Hyoid cartilage should be palpable at the level where soft tissue of jaw ends and most superior aspect of neck begins.
Dissect suprahyoid and infrahyoid muscles off of the midportion prior to removal, and never resect lateral to the greater horn of hyoid cartilage to ensure no injury to superior laryngeal nerve which enters lateral and inferior to the greater horn.
What if there has been significant infection of TGDC pre op?
Treat with systemic oral antibiotics (amoxicillin/clavulanate, or clindamycin) for 7–10 days and let infection “cool”.
Then expect and prepare for a more difficult dissection with poor tissue planes.
Be patient, elevate skin flaps and strap muscles, perform gentle dissection using a baby Jake, “peanuts”, which will help, remove all soft tissue outside of well-defined TGDC wall.
TGDC—What to do if there is a “fistula” and prior “drainage”?
Treat preoperatively as above.
Mark and make an elliptical excision surrounding the “fistula”, so that superior and inferior incisions are part of the skin flaps elevated.
Dissect superiorly following the fistula and all unhealthy tissue for an “en bloc” excision of entire fistula, all the way up to the mid portion of the hyoid cartilage.
Which masses will imaging be indicated and helpful? Which imaging studies? [5]
TGDC—Clinical diagnosis, no routine CT or ultrasound necessary
BCC—CT with contrast
Preauricular pit/fistula with recurrent swelling and infection—MRI face/neck with gadolinium
3rd BCC are rare, presents typically with neck swelling at level of thyroid gland and even abscess.
CT with contrast is helpful, but diagnosis is definitive when ENT performs microlaryngoscopy and finds a sinus opening at base of pyriform aperture.
CT with contrast may show air in the sinus and track from pyriform sinus to the neck.
Neck masses—CT with contrast, and/or MRI with gadolinium for solid tumors
Suspected infectious lymphadenitis—ultrasound ONLY for uncomplicated lateral neck, and CT if limited neck ROM as need to rule out retropharyngeal/para- pharyngeal phlegmon/abscess.
Pearls for lateral neck/branchial cleft anomalies?
Do not perform needle aspiration of suspected BCC (often perfectly “round” on CT with contrast).
Doing so creates a fistula to the outside and persistent drainage of serous mucous-like material, and will make future excision incredibly difficult.
Rare diagnosis for large lateral neck masses?
Castlemann’s disease, lymphoma, ganglioneuroblastoma, ganglioneuroma, neurofibroma fibrosarcoma.
To drain or not to drain?
Avoid I&D of acutely infected congenital masses like preauricular pit/cysts, TGDC and BCC whenever possible, but for acute lymphadenitis appropriate aggressiveness leads to fast recovery.
Stab incision of 3–4 mm at apex of “mass”, followed by curved mosquito clamps, gentle but fearless entry through capsule of lymph node, will release purulence.
If only “dishwater” is encountered, proceed to use clamp tips to manually debride all central necrosis, irrigate with saline or bacitracin solution, then can pack using 1⁄2 inch plain gauze or leave 1⁄4 in Penrose in for 12–24 hours.
Peri and postoperative considerations?
After I&D of cervical lymphadenitis, expect resolution of fever within 12 hours, often can discharge to home next day on oral broad spectrum antiotic for 3–5 days or less.
Excision of dermoid or preauricular lesions, and NTM excisional biopsies may go home same day.
Sistrunk procedures deserve overnight observation, removal of “rubber band” or Penrose drain the next morning and discharge to home.
How to treat post op fistula?
Depending on fistula, extent, moist to dry packing (1/4 in plain gauze in saline) changed twice a day by parents at home may or may not be an option.
If so, pack daily for 7–10 days until fistula heals by secondary intention.
If packing not indicated, clean area with gentle soap and water twice daily or more, cover loosely with gauze or telfa dressing.
Apply Desitin (diaper rash cream) to fistula open- ing 2–3 times daily for one week, often it can dessicate fistula and allow complete healing.
May add oral antibiotics if there is associated soft tissue erythema, inflammation, or purulent drainage from fistula.
When to consider re-excision of recurrent fistula?
If despite all efforts listed above, and period of reasonable observation a fistula persists consider MRI with gadolinium if no imaging was done preop.
When to ask for help from ENT colleagues?
—ANYTIME!
Discuss thyroglossal duct cysts.
Thyroglossal duct cysts and sinuses are the remnants of thyroid development occurring along the path of descent of the thyroid anlage from the foramen cecum to the neck in the midline.
It is the most common midline neck lesion in children and generally presents as an asymptomatic mobile mass that elevates with protrusion of the tongue or swallowing.
The most common indications for surgical excision is infection and risk of malignancy.
Surgical excision by Sistrunk procedure is the standard of care and is associated with minimal morbidity.
Long-term outcomes are excellent following complete excision with a low risk of recurrence.
What are thyroglossal duct cysts (TGDC) and sinuses (TGDS)?
Thyroglossal duct cysts are remnants of thyroid development.
The thyroglossal duct marks the course as the thyroid anlage descends from the foramen cecum, tracking anteroventral to the hyoid bone before resting at the thyroid cartilage.
TGDC result from failure of the thyroglossal duct to obliterate [1].
A TGDC with a skin opening (usually following infection with drainage) is known as TGDS.
What are the histological features of TGDC and TGDS?
TGDC are lined with respiratory columnar epithelium, squamous epithelium or both and contain microscopic foci of thyroid tissue within the cyst wall in 70% of cases [2].
More substantial thyroid tissue may be present within TGDC-resulting in hypothyroidism post excision if no other thyroid gland is present in the patient.
How common are TGDC?
TGDC are the most common midline neck lesions in the pediatric population.
TGDC are present in approximately 7% of adults worldwide with 2.2/100,000 population at risk per annum.
TGDC have a bimodal age distribution, presenting commonly in early childhood (more commonly among males) but also in adulthood in the fifth decade (more commonly females).
What are the clinical features of TGDC?
TGDC are typically found in four locations-at the base of the tongue or intra-lingual, in the midline of the neck with 20–25% supra-hyoid or submental, 15–20% thyro-hyoid and 45–65% sub-hyoid/supra-sternal [1].
Once infected, the sinus opening of the TGDS may be in the midline or slightly off midline with intermittent drainage.
On palpation, TGDC not previously infected are mobile cysts that elevate with tongue protrusion or swallowing, indicating their proximal fixation to the base of the tongue.
What are the symptoms associated with TGDC and TGDS?
TGDC most commonly present in children as asymptomatic midline lesions of the neck that enlarge over time but 10–28% of patients present with infected TGDC with painful, erythematous midline lesion. Once the cyst drains to the skin, the TGDS or fistula tract is formed with drainage.
Recurrent infections may occur.
Large, lingual TGDC may cause airway obstruction in infants and in adults; lingual TGDC may present with dysphagia and pharyngeal discomfort.
How can one distinguish TGDC from other cysts or masses of the neck?
Epithelial inclusion cysts (i.e. dermoid cysts) are also commonly found in the mid- line of infants and children.
Clinically, dermoid cysts may have a slightly more bright yellow colour compared to TGDC and should not move with swallowing.
Lymph nodes are also commonly found in the neck and also should not move with swallowing.
Type 2 branchial cleft cysts are usually situated laterally along the anterior border of the sternocleidomastoid muscle.
Ectopic thyroid tissue may also present as a mass in the midline above the thyroid cartilage due to incomplete descent and can be found anywhere along the thyroglossal duct.
How can one distinguish TGDS from other lesions with sinuses of the neck?
For TGDS, the sinus opening is generally in or near the midline and commonly near the hyoid, whereas type 2 branchial cleft sinus openings are localized in the lower lateral neck region in close relation to the clavicular head of the sternocleidomastoid.
What imaging is best for distinguishing TGDC from other neck cysts?
Ultrasound (US) is the best imaging modality.
Three US features most strongly predictive of TGDC are internal septae, irregular wall and presence of solid components [3].
Other helpful features include distance from the base of the tongue, location of the cyst relative to the hyoid bone, presence or absence of the tract, margin definition and intra-lesional Doppler flow.
Are there other investigations that can help with TGDC diagnosis?
CT and MRI imaging may also help to determine the diagnosis but these involve exposing children to ionizing radiation (CT) and/or general anesthetic/sedation (CT/MRI).
Nuclear medicine scans of the thyroid are not routinely performed in children but would reveal a “cold” nodule.
Routine thyroid function tests are not generally useful in the diagnosis of TGDC.
What complications commonly arise from thyroglossal duct remnants?
Infection is the most common complication and also the most common indication
for the surgical excision of TGDC.
Can TGDC become cancerous?
The incidence of cancer in TGDC is 1%.
Thyroid papillary carcinomas and follicular carcinomas have been found in TGDC in children and adults.
The cancer is usually completely excised with TGDC excision but thyroidectomy is recom- mended in adults age >45 years due to the risks of cancer recurrence [4].
What are the symptoms associated with a lingual TGDC?
Very large, lingual TDGC can be a cause of upper airway obstruction, presenting with stridor and/or dyspnea, even mimicking an upper respiratory tract infection.
More dramatically, there are case reports of asphyxia resulting in sudden deaths of infant and adults with the autopsies revealing the presence of the TGDC obstructing the upper airway at the epiglottis [5, 6].
What is the treatment for TGDC and TGDS?
Antibiotics are required to treat infected TGDC and TGDS.
Surgery remains the most definitive treatment option as complete excision of the entire thyroglossal duct remnant eliminates both the infection and cancer risks.
The standard of care is surgical excision of TGDC and TGDS using the Sistrunk procedure.
Simple excision of the cyst alone is associated with a 45–55% recurrence rate whereas the Sistrunk procedure is associated with <10% recurrence rate [1].
What are the steps in the Sistrunk procedure?
The Sistrunk procedure involves en bloc resection of:
– the cyst
– the central third of the hyoid bone and
– the thyroglossal duct from the cyst up to the base of the tongue.
More than one duct or tract may be associated with a single cyst, further emphasizing the need for clearly visualizing the cyst and tract(s) as the mobilization pro- ceeds through the midline.
What are the manoeuvres to optimize surgical management of TGDC?
Surgical considerations should include: pre-operative confirmation of the presence of an orthotopic thyroid (usually by US), extinguish any active infections prior to surgery (generally 3 months following infection), positioning the patient with a substantial under shoulder roll to allow good extension of the neck to aid exposure, nasal intubation to allow manual access into the oral cavity at the base of the tongue to push down during the cephalad dissection of the thyroglossal duct and inclusion of an ellipse of skin around the sinus opening of the TGDS to ensure complete TGDS excision.
What are the complications from surgery for TGDC/TGDS?
The most common complications following TGDC excision are wound infection, seroma, incomplete resection with recurrence, fistula, abscess, hematoma, hypoglossal nerve palsy (resulting in immobility of half of the tongue), hypothyroidism.
Less common but devastating complications include resection of the thyroid cartilage, airway compromise and severe hemorrhage requiring multiple transfusions.
Can TGDC/TGDS recur following surgery? What are the management options for recurrent TGDC/TGDS?
Recurrence affects approximately 10% of cases and generally occurs within 1 year of the initial surgery.
Recurrences manifest as persistent ± enlarging neck mass.
Re-excision should include the previous incision, incorporate any sinus openings, wider excision down to the thyroid isthmus between the strap muscles, and include the medial margins of the strap muscles and the remaining hyoid bone remnants.
Discuss branchial cleft and arch anomalies.
Branchial arch and cleft anomalies are congenital malformation of the first, second, third and fourth branchial arch and most frequently present as cysts, sinuses or fistulae in the auricular, mandibular or cervical areas.
Good knowledge of embryological development helps to understand the anatomy and relationships of branchial anomalies with surrounding structures, especially the laryngeal nerves (superior and recurrent) and vascular structures.
Diagnostic work up includes a good physical exam, ultrasonographical evaluation as well as MRI for cross-sectional imaging in more complicated cases.
Fistulogram and endoscopy can also be helpful, especially in third and fourth branchial cleft anomalies.
Surgical excision is usually the treatment of choice, on an elective basis.
If the cervical cyst, sinus or fistula are infected, incision and drainage and antibiotic treatment are recommended, followed by elective excision when the inflammation has resolved.
How many branchial clefts and arches are clinically relevant?
The face, neck, pharynx and multiple glandular component develop from branchial arches and clefts (pouches) in the human embryo.
There are six branchial arches that appear between the fourth and seventh week of gestation and form the precursors to the ear, muscles, blood vessels, bones, cartilage and glandular structures of the face, neck and superior thorax (see Table 44.1, reproduced with permission [1]).
The branchial arches give rise to an arch, a cleft and a pouch, forming ectodermal, mesodermal and endodermal structures, respectively.
The first 4 branchial arches and clefts are the most clinically relevant for congenital malformations of the head and neck for which pediatric surgeons are involved.
The fifth branchial arch is almost inexistent in humans. A branchial anomaly and its associated sinus usually lies caudally to the derivatives of the arch.
The malformations are therefore divided into the first to the fourth branchial anomalies, including arches and clefts.
