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.
What is the incidence of congenital branchial cleft anomalies?
Branchial anomalies are the second most common congenital malformations of the head and neck, after thyroglossal duct cysts, and encompass approximately 20% of all congenital head and neck masses.
Second branchial cleft anomalies are the most frequent subtype of anomalies amongst the branchial malformations.
There is a slight female preponderance and up to 20% of sinuses are bilateral.
How are the different branchial clefts defined and anatomically located?
The first branchial arch gives rise to the maxilla, and mandible as well as the exter- nal auditory structures, and two ossicles (incus and malleus).
The second branchial arch forms part of the hyoid bone, middle structures including the stapes and the supratonsillar fossa as well as the palatine tonsils.
The third branchial structures give raise to the greater horn of the hyoid bone, the thymus and the inferior parathyroid glands.
And the fourth branchial arch and cleft form the laryngeal cartilaginous structures, the superior parathyroid glands as well as the C-cells of the thyroid. See Table 44.1 for full details.
How does a first branchial cleft anomaly present?
First branchial cleft anomalies are rare.
The first branchial arch and cleft are involved in the development of the external ear, middle ear auditory tubes, as well as the incus and malleus.
Remnant of the first branchial cleft can present with lesions located between the external auditory canal and the submandibular area.
They can be mistaken for pre-auricular pits or sinuses.
The first cleft anom- alies may be closely associated with the parotid gland.
The first branchial clefts are divided into type 1 and type 2, type 1 being a duplication of the membranous external auditory canal and type 2 being of ectodermal and mesodermal origins and may include cartilage.
Type 2 lesions pass medial to the facial nerve and can present at pre-auricular, infra-auricular, or post-auricular locations.
They can present as masses, infected lesions, cysts, draining sinuses or otorrhea.
What work up is performed?
A careful physical exam of the lesion and its location is necessary.
Any sinus, mass, or cyst within the pre-, infra- or post-auricular area, as well as submandib- ular space should be suspicious for a first branchial anomaly.
An ultrasound and careful otoscopic evaluation should be performed.
Cross-sectional imaging for large lesion can be helpful to better delineate the relationship of the cyst or sinus with the facial nerve [2].
How is a first branchial cleft managed?
Surgical excision is the treatment of choice.
Careful dissection of the lesion is performed with special attention to the facial nerve.
Superficial parotidectomy has been recommended to decrease the risks of injury to the facial nerve. It is necessary to excise the skin or associated cartilaginous anomalies if the external auditory canal is involved.
Are there any complications or associated features to be concerned about?
Injuries to the facial nerve are the most feared complication during excision of the first branchial cleft anomalies.
Temporary facial nerve paralysis has been reported in up to 21% of cases, with 1% of permanent palsy.
Rare injuries to the parotid gland with salivary fistula can also happen.
If there was previous infection of the lesion, the scar tissue increases the difficulty of the dissection, as the planes might have been distorted.
How does a second branchial cleft present?
The second branchial cleft anomalies are the most frequent congenital branchial anomalies, with an incidence of 40% to 95%, varying between studies.
They usually present as an asymptomatic neck mass or sinus along the anterior border of the sterno-cleido-mastoid muscle.
The second branchial cleft anomalies can also present acutely after an upper respiratory infection with acute enlargement of the lesion.
Cystic lesions are more frequent than sinuses.
Sinuses can present with a small opening draining clear fluid, near the base of the neck, anterior to the sterno-cleido-mastoid muscle.
Sinuses are more frequent on the right side and bilateral sinuses can be found in up to 10 percent.
Less common symptoms include odyno- or dysphagia, stridor or cranial nerve palsies in case of infected lesions [2, 3].
What work up is performed?
A careful clinical exam of the neck is critical.
Sinuses and cyst are found anterior to the sterno-cleido-mastoid muscle.
Sometimes the tract can be palpated going cephalad.
Cysts are usually mobile and non-tender.
Some clear fluid can sometimes be expressed from sinuses, as they communicate with the tonsillar fossa.
Ultrasonography of the neck is recommended as initial imaging, even though a draining sinus in the correct location narrows the diagnosis.
For large or symptomatic lesions, an MRI may better delineate the cyst or sinus and allow for better surgical planning.
Rarely a sinogram can be performed but is currently more of historical interest [2].
How is a second branchial cleft managed?
As branchial clefts can become infected, and there is a reported potential long-term risk of malignant transformation, elective excision of second branchial cysts and sinuses is recommended.
The procedure is performed under general anesthesia and the head slightly turned to the contralateral side.
The sinus is excised with the skin opening and some surgeons use a small catheter, probe or dye to be able to follow the track cephalad.
Good knowledge of the embryology and neck anatomy is required.
Second branchial cleft sinuses or cysts track cephalad and end up in the tonsillar fossa, after having crossed the internal carotid artery antero-superiorly to the carotid bifurcation.
Wide skin preparation is mandatory and sometimes a second counter-incision is needed for safe excision of the sinus [3].
Are there any complications or associated features to be concerned about?
Incomplete resection increases the risk of recurrence or infection.
The risk of injuring neck structure is low if the dissection is accomplished close to the sinus tract.
There is a reported low risk of malignant transformation in adults.
How does a third branchial cleft anomaly present?
Third branchial cleft anomalies have an incidence of less than 10% of all branchial clefts and extend medially between the bifurcation of the internal and external carotid arteries, lateral to the pharyngeal wall.
They are more commonly found on the left side in the lower portion of the anterior neck.
Third and fourth branchial cleft anomalies are very similar to second branchial cleft anomalies externally with a cutaneous opening in the supraclavicular area; however, internally, the third and fourth enter the pharynx through the pyriform sinus below the hyoid bone.
Both are distinguished anatomically by their relationship to the superior laryngeal nerve with third pharyngeal cleft anomalies being above and fourth pharyngeal cleft anomalies being caudal to the superior laryngeal nerve.
Anatomically, the third branchial cleft remnants pass through the thyrohyoid membrane cephalad to the superior laryngeal nerve and open into the upper piriform sinus.
Third branchial cleft cysts present in the posterior cervical space more frequently, pos- terior to the sternocleidomastoid muscle as a painless, fluctuant mass that may enlarge and become tender if infected.
Abscesses in the posterior triangle of the neck should be considered as an infected third branchial cleft cyst.
Recurrent episodes of abscess can also promote thyroiditis [4].
What work up is performed?
There are many different forms of presentation of third branchial anomalies including fistulas, cysts or sinuses.
A classic third branchial fistula opens in the pyriform sinus and exits at the skin, which makes it an epithelized tract.
A cyst has no opening and sinus has an opening in direction of skin or pyriform sinus but not both.
Repeated procedures of drainage and incision may convert a sinus to a fistula [2, 3].
The diagnostic investigation should include a careful physical exam, and an Ultrasound.
Possible further investigation with magnetic resonance imaging (MRI) can be helpful to better delineate the anatomy.
Further fiberoptic endoscopy could be necessary to identify the entry point of a third and fourth branchial sinus at the level of the pyriform sinus.
In that case, the orifice of the sinus or fistula can be identified, and cannulation or injection of the tract can be helpful for surgical dissection.
How is a third branchial cleft anomaly managed?
Elective surgical resection is necessary to treat third branchial cleft anomalies.
As mentioned above, upper endoscopy can be helpful to identify the internal pyriform sinus opening.
The operation is performed under general anesthesia and the neck is widely prepped.
The dissection must respect the anatomic structures in order to avoid any damage and perform a complete resection of the cyst, sinus or fistula to its origin.
Very careful attention to the superior and recurrent laryngeal nerves are necessary to prevent injury, as these nerves are closely associated with a third branchial cleft anomaly.
Rarely, these cysts can extend into the chest.
Previous infections can make the dissection more difficult, secondary to scar tissue.
If the cyst or sinus is infected, drainage and antibiotic therapy are recommended followed by an elective excision once the infection and inflammation have resolved.
Are there any complications or associated features to be concerned about?
During the dissection, injury to the superior and recurrent laryngeal nerves has been documented.
Patients are also at risk of recurrence of cervical abscesses and mediastinitis, more commonly observed in incomplete excision of the sinus tract or cyst.
How does a fourth branchial cleft anomaly present?
A fourth branchial cleft fistula/sinus tract arises from the apex of the pyriform sinus and descends inferiorly to the mediastinum in the path of the tracheo-esophageal groove.
Fourth branchial anomalies can therefore present as lower cervical masses as third branchial cysts, but also with thyroiditis, dyspnea or dysphagia and rarely with chest pain.
Similarly to the third branchial anomalies, fourth branchial malformations are more frequent on the left side and their overall incidence is 1–4% of all congenital neck anomalies.
The fourth branchial sinus runs caudal to the superior laryngeal nerve and over the recurrent laryngeal nerve [4].
What work up is performed?
The diagnostic work up should include a careful physical exam, and an Ultrasound.
Possible further investigation with magnetic resonance imaging (MRI) can be helpful to better delineate the anatomy, especially if one suspects an extension into the chest.
Further fiberoptic endoscopy could be necessary, similarly to the third branchial anomalies to identify the entry point of a sinus at the level of the pyriform sinus.
In that case, the orifice of the sinus or fistula can be identified, and cannulation or injection of the tract can be helpful for surgical dissection.
How is a fourth branchial cleft anomaly managed?
Most of fourth branchial anomalies present as a cervical inflammatory process located at the left side, and half of them have associated acute suppurative thyroiditis or recurrent neck abscesses.
Elective surgical excision is recommended after drainage and treatment of the infection with resolution of the inflammation.
Fiberoptic endoscopy with possible catheterization of the fistula can facilitate the dissection.
Are there any complications or associated features to be concerned about?
Fourth arch anomalies with thyroiditis and abscess formation may require ipsilateral hemithyroidectomy for complete excision of the tract as well as partial resection of the thyroid cartilage to provide adequate exposure of the pyriform sinus.
As for third arch anomalies, careful attention to the recurrent laryngeal nerve is recommended, to prevent its injury.
As fourth branchial anomalies may extend into the chest, the surgeon and anesthesiologist need to be prepared for that possibility.
Are there any special considerations—syndromes in branchial cleft anomalies and their treatment?
Common syndromes associated with branchial cleft anomalies include Branchio-oto-renal syndrome (BOR), Treacher Collins Syndrome, DiGeorge Anomaly, Pierre Robin Sequence, Goldenhar’s Syndrome and Hemifacial Microsomia [5].
BOR syndrome is an autosomal dominant disorder and is characterized by branchial arch anomalies (branchial clefts, fistula, cysts), hearing impairment (malformations of the auricle with preauricular pits, conductive, or sensorineural hearing impairment), and renal malformations (urinary tract malformation, renal hypoplasia, or agenesis, renal dysplasia, renal cysts).
Treacher Collins Syndrome (mandibulofacial dysostosis, Franceschetti-Zwahlen syndrome) is an autosomal dominant genetic disorder, characterized by bilateral, symmetric abnormalities of first and second branchial arch structures.
DiGeorge Anomaly is a congenital disorder of the third and fourth branchial pouches in which there is abnormal development of the thymus and the parathyroid glands.
Pierre Robin sequence is characterized by micrognathia, glossoptosis, and a U-shaped cleft soft palate.
Goldenhar’s Syndrome and Hemifacial Microsomia are also called oculo-auriculo-vertebral syndrome and the anomaly is characterized by impaired development of eyes, ears (with or without hearing loss), lip, tongue, palate, mandible, maxilla and deformations of the teeth structures.
Recurrent cervical abscesses, on the lateral and posterior side, as well as thyroiditis in children associated with a cervical inflammatory process should raise suspicion about congenital branchial anomalies.
The most important recommendation is a correct location of the lesion, including sinus, cyst or fistula that therefore define the anatomy and allows the classification of a brachial cleft defect.
What is torticollis?
The term ‘Torticollis’ is derived from two Latin root words ‘tortus’ and ‘collum’ that together mean ‘twisted neck’. Torticollis is not a specific diagnosis but rather a clinical sign of ipsilateral head tilt with contralateral rotation [1].
Although it has been known since ancient times, it was first defined by A. H. Tubby in 1912. Interestingly, some portraits and statues of Alexander the Great depict him with his head tilted or twisted, which has led some medical researchers to believe he suffered from ocular torticollis.
What is congenital muscular torticollis?
There are many presentations of torticollis, however, the most prevalent is congenital muscular torticollis (CMT).
CMT is defined as a musculoskeletal deformity observed at birth or infancy, characterized by a persistent head tilt toward the involved side with the chin rotated toward the contralateral shoulder.
Classically, the sternocleidomastoid muscle (SCM) in involved.
How common is congenital muscular torticollis?
Historically, the incidence of CMT was reported to be 0.3–2% with overall incidence that can be as high as 1 in 250 live births. It is the third most common congenital pediatric musculoskeletal deformity, behind hip dysplasia and talipes equinovarus.
What are 3 different types of congenital muscular torticollis?
Congenital muscular torticollis can be divided into 3 groups based on presentation:
Group 1: Infants with sternocleidomastoid tumor. This group consists of torticollis with range of motion limitations and a palpable pseudotumor or swelling in the body of the SCM. The pseudotumor, also called fibromatosis colli, is a hard, immobile mass within the substance of the SCM and is noted at birth. It is usually located in the middle to lower third of the sternal portion of the SCM. This type occurs in 33–47% of cases.
Group 2: Muscular torticollis. This group consists of torticollis with limitations in active and passive range of motion secondary to tightness or contracture of the SCM, but no palpable intramuscular mass is present
Group 3: Postural torticollis. This group includes patients with clinical torticollis, in which there is no presence of SCM mass and no passive range of motion restrictions [2]
Describe hypotheses for possible causes of congenital muscular torticollis.
The exact cause of CMT is still unknown, however, there are a variety of hypotheses.
Many revolve around in utero positioning or trauma during delivery. One the- ory suggests that CMT is caused by a congenitally shortened SCM torn at birth, resulting in the formation of a hematoma that underwent fibrous contracture. Ischemia within the SCM produced by possible venous occlusion has also been posited.
Another hypothesis is intrauterine or perinatal compartment syndrome involving the SCM.
Less likely causes have also been described, such as infec- tion and heredity.
Despite these hypotheses insufficient evidence has been found to definitively identify a cause for CMT.
What are the three strongest negative prognostic factors for congenital muscular torticollis treatment outcomes?
- Sternocleidomastoid mass present
- > 15 degrees passive range of motion deficit in cervical rotation
- Physical therapy treatment started >3 month of age.
These three factors contribute to patients requiring a longer duration of physical therapy, having an increased need for surgical intervention, and having an overall poorer outcome
If left untreated, what sequelae can develop from congenital muscular
torticollis?
Secondary sequelae from the presence of CMT include plagiocephaly and facial asymmetry. This is primarily caused by the forces of gravity pulling unevenly on a baby’s tilted head, causing a flattened appearance on one side of the skull or face. This emphasizes the importance of early identification and treatment to prevent the development of these secondary issues.
What are non-muscular causes of torticollis?
It is imperative that congenital muscular torticollis is differentiated from other congenital or acquired types of torticollis [3]. There are a variety of known non-muscular causes of torticollis in children and can include:
• Osseous: includes Klippel-Feil anomalies, scoliosis, clavicle fracture, and atlantoaxial rotatory subluxation
• Ocular: includes 4th cranial nerve palsy and resulting strabismus, Brown’s syndrome, and gaze dysfunction
• Neurologic: includes brachial plexus palsies and central nervous system lesions
• Otologic: includes saccular dysfunction
• Inflammatory: includes Grisel’s syndrome after adenoidectomy
• Gastrointestinal: includes Sandifer syndrome.
How common is non-muscular torticollis in children?
Non-muscular causes were thought to be rare compared to CMT, however, recent studies have shown this may be occurring more frequently than initially thought; showing that approximately 20% of torticollis is non-muscular in nature. This highlights the need for providers to be well educated in the differential diagnosis for torticollis, as misdiagnosis can cause a delay in appropriate treatment.
In the 2018 clinical practice guidelines for the management of congenital muscular torticollis, why is there such an emphasis on early referral to physical therapy and a physician for evaluation?
Early intervention has shown to require a shorter duration of physical therapy and a higher chance of reaching complete resolution of the torticollis [4].
More than 98% of infants with torticollis treated before 1 month of age recover by 2.5 months of age.
Infants treated between 1 and 6 months usually require about 6 months of treatment.
After that point, therapy will take closer to 9 months, and it is less likely that the torticollis will be fully resolved, resulting in the need for surgical intervention.
Early identification, referral, and intervention are key to obtaining the best possible outcome for the child.
What is recommended if the patient does not progress with 4–6 weeks of intensive physical therapy?
Recommendations are for therapist to refer to a physician for re-evaluation and assessment for non-muscular causes of the torticollis.
What is the preferred radiological modality in the diagnosis of torticollis?
Due to its low cost and non-invasive nature, ultrasonography can be used to aid in the diagnosis of CMT.
For CMT, ultrasound has been shown to correlate with the severity of disease but is not sensitive enough to determine changes in the muscular architecture [5].
Other imaging modalities such as computerized tomography (CT) and magnetic resonance imaging (MRI) are not necessary for the initial work-up and management of CMT, however they may be useful when investigating non-muscular causes of torticollis.
Describe the ultrasound features of normal sternocleidomastoid muscle.
The normal SCM on ultrasound presents as a hypoechoic mass with echogenic lines, indicating muscle fascicles running throughout its length.
What are characteristic features on ultrasound for patients with
congenital muscular torticollis and sternocleidomastoid pseudotumor?
The presence of a SCM pseudotumor affects not only the size of the muscle, but also its signal intensity. For patients with CMT, the involved SCM tends to be larger and will appear more hyperechoic on ultrasound.
Describe the primary therapy modality for congenital muscular torticollis.
Physical therapy is the primary treatment modality for CMT. The 2018 Clinical Practice Guidelines outline five main areas of therapy intervention: passive neck range of motion exercises, cervical and trunk muscle strengthening, development of an infant’s symmetrical movement, environmental adaptations, and parent edu- cation. The frequency of treatment can be based on the age at presentation, the presence of a fibrotic mass in the sternocleidomastoid, and the infant’s neck rota- tional deficit.
16.
What medical therapies can be used as an adjunct therapy for congenital muscular torticollis?
Botulinum toxin type A has been shown to be beneficial as an adjunct therapy in patients with CMT and is to be used in conjunction with physical therapy. Systemic medical therapies such as muscles relaxants have shown no benefit and are to be avoided [6].
In the event of medical and therapy failure, what surgical interventions can be employed to treat congenital muscular torticollis?
Surgical release of the sternocleidomastoid muscle is used mainly in cases of severe CMT that are unresponsive to more conservative therapies. Surgical inter- vention involves a unipolar or bipolar approach. For the unipolar approach, the sternocleidomastoid muscle is released from either its mastoid or sternum/clavic- ular insertions, whereas a bipolar approach releases the SCM from both insertion points.
Which patients tend to require surgical intervention?
- Persistent sternocleidomastoid muscle contracture limiting head movement
- Persistent sternocleidomastoid muscle contracture accompanied by progressive facial hemihypoplasia
- Torticollis in untreated children older than 12 months of age.
List some potential complications of surgical management of congenital muscular torticollis.
• Hematoma
• Wound dehiscence or infection
• Non-resolution of torticollis
• Vascular injury to the carotid artery, internal jugular vein, or brachiocephalic
artery
• Neural injury to the spinal accessory nerve or the brachial plexus.
What are the histologic findings of congenital muscular torticollis?
Histologic studies of the resected surgical specimens have demonstrated edema,
degeneration of the SCM muscle fibers, and fibrosis.
The most common branchial anomalies are:
A. First branchial anomalies
B. Second branchial anomalies
C. Third branchial anomalies
D. Fourth branchial anomalies
E. Fifth branchial anomalies
B. Second branchial anomalies
Features of second branchial cyst include all except:
A. Painless.
B. Gradually increases in size.
C. Presents in upper part of posterior triangle of neck.
D. Usually presents at three to six years of age.
E. Rarely presents with stridor, sore throat and dysphagia
C It presents as swelling in upper part of anterior triangle of neck.
Syed/MCQ
Regarding cleft lip and cleft palate, which of the following is false?
A. These constitute 80 percent of all cleft types.
B. Incidence of cleft lip is more than cleft palate.
C. Left-sided cleft lip is more common than right-sided.
D. Aetiology of clefts is multifactorial.
E. All of the above are false.
E All statements A, B, C and D are true.
Syed/MCQ
Regarding cleft palate, which of the following statements is true?
A. Development of primary palate related to structures posterior to incisive foramen.
B. Palatal process develops bilaterally during tenth to twelfth week of gestation.
C. Frequency in general population is 0.2 percent.
D. Repair is done during 3–4 months of age.
E. Delayed repair is indicated in Pierre Robin sequence.
E
Cleft palate is usually repaired in 6–18 months.
In Pierre Robin sequence, repair should be delayed because of airway obstruction.
Development of primary palate (prepalate) is related to structures anterior to incisive foramen (i.e., the face, lips, premaxilla and upper four incisor teeth).
Secondary palate is related to structures posterior to incisive foramen (i.e., hard palate, soft palate and maxillary teeth).
Primary palate develops during fourth to seventh week of gestation and secondary palate during sixth to seventh week.
Frequency of cleft palate in general population is 0.02 percent.
Syed/MCQ
Complications of cleft palate include all except:
A. Otitis media.
B. Respiratory tract infection.
C. Meningitis.
D. Turbinate atrophy.
E. Brain abscess.
D
Turbinate undergoes hypertrophy.
Syed/MCQ
Regarding Millard’s procedure of cleft palate repair, which of the following is not true?
A. Rotation of medial segment.
B. Advancement of lateral segment.
C. It is flexible technique which can be modified during course of operation.
D. The cupid bow is not preserved.
E. Scar lies in good position which greatly facilitate secondary correction.
D
Millard’s procedure is a rotational (medial) and advancement (lateral) procedure, in which cupid bow is preserved.
Syed/MCQ
Regarding salivary gland, which of the following statements is false?
A. In solilithiasis, parotid gland is involved in 90 percent of cases.
B. Majority of submandibular glands are radio-opaque.
C. Ninety percent of parotid gland stones are radiolucent.
D. Pleomorphic adenoma does not account for more than 50 percent of parotid tumours.
E. Most parotid haemangiomas regresses within two years.
A
In solilithiasis, submandibular gland is involved in 90 percent of cases, 90 percent of which are radio-opaque.
In parotid tumours 32 percent endothelial tumours (haemangioma and lymphangioma), 40 percent are benign epithelial tumours (32 percent are benign pleomorphic adenoma), and 27 percent are malignant epithelial tumours.
Syed/MCQ
Regarding craniofacial anomalies, all of the following are true except:
A. In craniofacial macrosomia, parotid gland is hypoplastic or absent.
B. Cranial cleft is characterised by orbital hypotelorism.
C. Treacher Collins syndrome is mandibulofacial dysostosis.
D. In Treacher Collins syndrome, there is bilateral symmetrical involvement.
E. Zygomatic bone is absent in Treacher Collins syndrome.
B
Cranial cleft is characterised by orbital hypertelorism (increased infraorbital distance).
Syed/MCQ
Which statement is false regarding nonsyndromic craniocynostosis?
A. Scaphocephaly develops by premature closure of multiple sutures.
B. Plagiocephaly develops by premature closure of unilateral coronal suture.
C. Brachiocephaly develops by premature closure of bilateral coronal sutures.
D. Trigonocephaly develops by premature closure of metopic sutures.
E. After linear strip craniotomy, incidence of refusion is high.
A
Scaphocephaly is premature closure of sagittal sutures.
Syed/MCQ
Regarding syndromic craniocynostosis, which of the following is false?
A. Crouzon syndrome is autosomal dominant.
B. Excorbism is a feature of Crouzon syndrome.
C. Apert’s syndrome has some features like those of Crouzon syndrome.
D. In Crouzon syndrome, mental retardation is significant.
E. Symmetrical syndactyly is noted in Apert’s syndrome.
D
In Crouzon syndrome, normal intelligence is seen.
Syed/MCQ
Regarding tracheostomy, which is not true?
A. Severe epiglottitis is an indication of tracheostomy.
B. Position during tracheostomy, the neck is hyperextended.
C. Vertical incision is made over trachea at first and second tracheal ring.
D. It is essential to leave a gap around the tube to avoid post-operative surgical emphysema.
E. Persistent tracheal fistula after prolonged tracheostomy is a known complication.
C
Vertical incision is made in the trachea through second, third and fourth tracheal ring.
Syed/MCQ
Causes of torticollis includes all of the following, except:
A. Sternocleidomastoid tumour.
B. Cervical hemivertebrae.
C. Retropharyngeal abscess.
D. Pyloric stenosis.
E. Sandifer syndrome.
D
Pyloric stenosis is not cause of torticollis.
Sandifer syndrome is a combination of gastroesophageal reflux with spastic torticollis and dystonic body movements.
It is a hypothesis that the positioning of head provides relief from abnormal discomfort caused by acid reflux.
Syed/MCQ
Regarding pathophysiology of sternocleidomastoid tumour/torticollis, all of the following are true except:
A. Breach deliveries are seen in 20–30 percent of cases.
B. It resolves spontaneously in the vast majority of cases.
C. Plagiocephally is a known complication, seen by gravity effect.
D. Bilateral facial hypoplasia has been seen.
E. Fibrous replacement of muscle bundle has seen.
D
Unilateral facial hypoplasia is seen by progressive asymmetry by unilateral immobilisation of face.
Syed/MCQ
Regarding operative treatment of sternocleidomastoid tumour/torticollis, all of the following are true except:
A. Indication of surgery is sternocleidomastoid tightening, limiting head rotation beyond 3 months of age.
B. Incision is given 1 cm above the sternal and clavicular head of sternocleidomastoid muscle.
C. Full range of movement normally achieved within one week of surgery.
D. Recurrent torticollis is a known complication.
E. Patient should be followed after surgery until torticollis has been resolved completely.
A
Indication of surgery is persistent sternocleidomastoid tightening, limiting head rotation beyond 12–15 months of age.
Other indication is progressive faecal hypoplasia and presentation in children older than one year of age.
Syed/MCQ
The most common brachial anomalies are:
A. First branchial anomalies.
B. Second branchial anomalies.
C. Third branchial anomalies.
D. Fourth branchial anomalies.
E. Fifth branchial anomalies.
B
Ninety percent of branchial anomalies are second type.
Syed/MCQ
Regarding thyroglossal duct cyst, which one is false?
A. Most common congenital midline neck mass .
B. Enlarged lymph node is one of the differential diagnoses.
C. Excision is accompanied with excision of central part of hyoid bone.
D. One percent develop carcinoma, mostly papillary.
E. It is derivative of second branchial arch.
E
Thyroglossal cyst is not derivative of second branchial arch.
It is formed from persistence of thyroglossal duct.
In other words, this anomaly is caused by a tract of thyroid tissue along the pathway of the embryological migration of the thyroid gland from the base of the tongue to neck.
Syed/MCQ
With regards to solitary thyroid nodule, all are false except:
A. It is common in children as compared to adults.
B. When present they are more likely to be benign.
C. Most are well differentiated papillary carcinoma.
D. Cancers occur most frequently between 5 and 10 years of age.
E. Boys are affected more than girls.
C Most are well differentiated papillary carcinoma.
Solitary thyroid nodule is rare in children. When present they are most likely malignant.
Cancer most frequently occurs at 15–19 years of age.
Girls are affected more than boys.
Syed/MCQ
Which of the following is true regarding thyroglossal cyst?
A It is due to entrapment of epithelial tissue during the descent of the thyroid gland.
B The gland passes inferiorly through the midsection of the cricoid.
C Less than 5% of cysts are found lateral to the midline.
D The primordial thyroid forms from the third branchial arches.
E The most common presentation is with a discharging sinus.
A
The thyroid gland develops as an outpouching of the floor of the embryonic pharynx at the foramen caecum and descends through the hyoid bone to its final position in the anterior neck.
Entrapped epithelial tissue left behind during this descent gives rise, in later life, to a thyroglossal cyst.
More than 75% of cysts are diagnosed during childhood.
The most common presentation is with an asymptomatic midline lump rather than the less common discharging sinus.
At least 25% of cysts are found lateral to the midline and may be intrathyroid, intralingual, suprahyoidal or suprasternal.
SPSE 1
Which of the following is true regarding a preauricular sinus?
A Routine renal ultrasound is indicated because of high incidence of associated anomalies.
B It is more common in Caucasians than African Americans.
C The risk of recurrence after excision surgery is less than 5%.
D It is not found in patients with Treacher Collins’s syndrome.
E There is a male predominance.
C
The incidence of associated renal anomalies in patients with preauricular sinuses is very low and most authorities would not require ultrasound in such children.
The anomaly is more common in African Americans than Caucasians and occurs with no sex preponderance.
It is common in various syndromes, not the least the Treacher Collins’s and branchio-oto-renal syndromes.
Surgical excision is indicated for frequent infection and is associated with a recurrence rate in major studies approaching 10%.
SPSE 1
Which of the following is not true regarding piriform sinus fistula?
A It occurs on the left in over 90% of cases.
B It may lead to acute thyroiditis.
C The piriform sinus fistula develops from the second branchial pouch.
D It may be effectively treated with trichloracetic acid.
E It should be considered in the differential diagnosis of acute deep neck infection.
C
Piriform sinus fistulas are a very rare, but important cause of acute deep neck infection.
There is debate regarding the exact embryological origin, whether due to third or fourth branchial pouch problems.
The condition occurs almost uniformly on the left side.
The anatomical connection to the thyroid gland leads to marked inflammation of the gland, infection probably being reduced in severity because of the high iodine content of the tissue and the protective effect of the capsule.
Surgical excision is recommended but because of the associated risks of surgery, some fistulas are effectively treated using injection of 40% trichloracetic acid into the mouth of the sinus.
SPSE 1
Which of the following is a true statement regarding branchial arch embryology?
A Third cleft anomalies are the most common.
B Anomalies of first pouch typically have their external orifice inferior to the ramus of the mandible.
C Overall, cleft anomalies are more commonly right-sided.
D Sinuses of third arch anomalies common pass anterior to the carotid bifurcation up to the piriform fossa.
E Fourth cleft anomalies, although rare, occur more frequently than first cleft anomalies.
B
Branchial arch anomalies most frequently occur on the left and most commonly in those derived from the second arch.
The uncommon third arch sinus passes posterior to the carotid bifurcation to its drainage in the piriform fossa of the pharynx.
First arch anomalies, although very rare do drain inferior to the ramus of the mandible.
There is debate as to whether fourth arch anomalies actually exist at all.
SPSE 1
Which of the following is true of atypical mycobacterial sinus formation in the neck?
A The intestinal tract is the primary route for Mycobacterium avium infection.
B Mycobacterium scrofulaceum is associated with lymphadenitis in immunocompetent children.
C Disseminated atypical mycobacterial disease is the second most common opportunistic infection in children with HIV infection.
D All of the above.
E None of the above.
E
Atypical mycobacteria are a problematic cause of recurrently infected and discharging lymph node abscesses in the neck often leading to chronic sinus formation.
The common organisms encountered are M. avium and M. scrofulaceum, which most frequently enter the body via the enteral route.
Such infections present significant risk for immune compromised children, in particular those with HIV.
SPSE 1
Which one of the following statements about cystic lymphatic obstruction is not true?
A It may be because of abnormalities of VEGF-C and its receptors.
B It is strongly associated with genetic abnormalities.
C It is most commonly seen on the right side of the neck.
D If occurring only in the third trimester of pregnancy, genetic abnormalities are rare.
E It may be effectively treated using intralesional bleomycin.
C
Cystic lymphatic malformations are variously described as lymphangioma, cystic hygroma and lymphatic obstruction sequence and occur because of a combination of lymphatic obstruction, failure of connection to the venous circulation and abnormalities of the VEGF-C endothelial growth factor and its receptors.
It is most commonly related to the lymphatic duct on the left side of the neck and has a strong association with genetic abnormalities, particularly trisomy 13, 18 and 21 if noted early in pregnancy.
Interestingly, cystic hygroma occurring only in the third trimester virtually never shows this genetic anomaly.
Surgical treatment is fraught with difficulty and conservative approaches have been tried, with varying success using sclerosants such as OK-432, bleomycin, alcohol and even fibrin sealant.
SPSE 1
The preoperative workup for a child with a midline cyst should include:
A ultrasound scan of the thyroid gland
B fine needle aspiration
C methylene-diphosphonate isotope scan
D serum thyroid antibody-level measurement
E all of the above.
A
There is debate in the literature regarding the value of any investigation of a clinically euthyroid child with a suspected thyroglossal cyst.
It would be reasonable to include at the very least ultrasound examination of the thyroid gland.
Some practitioners persist in assessing thyroid function with serum thyroid-stimulating hormone (TSH) levels and even radioactive 123I scans.
Fine needle aspiration is contraindicated in children and antibody levels have no place in the assessment of such lesions.
SPSE 1
Which of the following is not true regarding bronchogenic neck cysts?
A They are congenital malformations of ventral foregut development.
B They may be directly connected to the tracheobronchial airway.
C They usually present with sinus tract formation and external drainage of purulent material.
D They have a 4 : 1 male-to-female ratio.
E They are lined by ciliated pseudostratified columnar epithelium.
C
Bronchogenic cysts form as a result of abnormal budding of the bronchial tree during embryogenesis.
The connection with the tracheobronchial tree is usually lost but the cellular lining directly reflects their respiratory origin: ciliated pseudostratified columnar epithelium.
There is a significant male predominance (4 : 1).
The majority of cervical lesions are asymptomatic and present not with discharge but due to coincidental factors including size.
Sinuses are uncommon in bronchogenic cysts.
SPSE 1
Which of the following statements is true with respect to surgical removal of a branchial arch remnant?
A The recurrent laryngeal nerve is at risk as it crosses through the carotid bifurcation with the fistula.
B The midsection of the hyoid bone must be excised to ensure low recurrence rates.
C The facial nerve must be formally identified when excising first arch anomalies.
D The piriform fossa must be explored to facilitate complete excision of second pouch anomalies.
E The accessory nerve is encountered at the medial border of sternocleidomastoid with the fistulous opening of second arch.
C
Intimate knowledge of the anatomy of this region is required before undertaking surgery.
The facial, hypoglossal, recurrent laryngeal and spinal accessory nerves are at most risk and should be formally identified.
The facial nerve lies close to the auditory canal and is at risk during first arch surgery.
The recurrent laryngeal nerve lies principally in the tracheo-oesophageal groove and the accessory nerve is at risk if surgery extends to the posterior border of sternocleidomastoid.
The internal opening of the second arch sinus is commonly in the posterior tonsillar fossa.
The hyoid bone is excised during the Sistrunk procedure for excision of thyroglossal cysts.
SPSE 1
The differential diagnosis of an isolated midline neck cyst does not include:
A branchial arch cyst
B dermoid cyst
C lymph node
D thyroglossal cyst
E cervical cleft.
A
Because of the laterality of embryological origin, branchial cysts are never midline.
An isolated prehyoid lymph node and a dermoid cyst must be included as part of the differential, the diagnosis often being made only during surgery.
Both may be firmly adherent to the hyoid bone, due to inflammation, and thus move with tongue protrusion.
The sticky, honey-like contents of a thyroglossal cyst are pathognomonic.
Congenital midline cervical cleft is a rare condition usually presenting as a cleft of pink tissue in the anterior midline neck, often with an associated protuberance of skin superiorly and a blind sinus tract inferiorly.
SPSE 1
