Introduction
Head and neck lesions in children can be divided into infectious, traumatic, neoplastic, or congenital categories. Benign neoplasms such as vascular anomalies or lymphatic malformations are discussed elsewhere in this text. Primary or metastatic neoplasms of the head and neck, benign and malignant processes of the thyroid and parathyroid, as well as head and neck trauma, are also discussed elsewhere. Common congenital head and neck cysts and sinuses as well as inflammatory lesions will be reviewed in this chapter.
Lesions of Embryonic Origin
Congenital cysts and sinuses in the neck result from embryonic structures that have failed to mature or have persisted in an aberrant fashion. , Both proper diagnosis and appropriate treatment depend on a detailed knowledge of the embryologic origin and differentiation of the head and neck structures. , This knowledge is particularly important because complete resection of cartilaginous remnants, remnants of the branchial arch and cleft structures, and midline fusion abnormalities is needed to avoid recurrence. Congenital lesions of the head and neck include thyroglossal duct cysts, preauricular pits and sinuses, branchial cleft anomalies, dermoid cysts, and median cervical clefts.
Branchial Cleft Anomalies
Branchial anomalies comprise roughly 30% of congenital neck masses. They are equally common in males and females and present in childhood or early adulthood. Proper nomenclature should be used for descriptive purposes. Fistulae connect two open spaces; notably, they connect the pharynx or external auditory canal with the skin. Sinuses have one opening either within the external auditory canal, the pharynx, or an opening facing the skin, all with a blind-ending pouch. In addition, a cyst has no external or internal opening. Branchial cleft anomalies can, therefore, extend through the soft tissue and completely into the pharynx or external auditory canal, or they can end in a short blind pouch with no deeper extension.
A thorough understanding of the embryonic branchial anatomy is important in the surgical management of these lesions. During the second week of gestation, the branchial apparatus develops, and by weeks 4–8, four pairs of arches separated by clefts are discernible. Each arch is associated with an artery, nerve, skeletal structures, and muscles ( Table 69.1 ). Attention to the structures of each arch, cleft, and pouch triad is essential to prevent iatrogenic injury and to ensure complete resection. Each arch and cleft pair are formed without a true connection between the ectodermal clefts and the endodermal pouches, with the final, mature structures being completely formed by the pharyngeal arches with obliteration of the clefts and pouches. The first branchial cleft invaginates to form the external auditory canal, and malformations of this apparatus may lead to a duplication of structures. As the second branchial arch grows caudally, it covers the second, third, and fourth branchial clefts and the third and fourth arches. Incomplete obliteration and the persistence of trapped epithelium ultimately results in a branchial anomaly. Histologically, branchial fistulae and sinuses are lined with a respiratory epithelium and cyst squamous epithelium. Other epithelial, ectodermal, or mesenchymal structures can also be found, including lymphoid tissue, sebaceous or salivary secreting tissue, or hair follicles ( Fig. 69.1 ).
Table 69.1
Branchial Cleft Anomalies are Composed of a Named Vascular Structure, Nerve, Cartilage, and Muscle Owing to Their Inclusion of All Three Embryologic Germ Layers
| Arch | Vascular Structures | Nerve | Skeletal Structure | Muscles |
|---|---|---|---|---|
| I | Maxillary artery | Trigeminal (CN V 1 and V 2 ) |
Meckel’s cartilage:
|
Muscles of mastication and:
|
| II |
Stapedial artery
Caroticotympanic artery |
Facial nerve (CN VII) |
Reichert’s cartilage:
|
Muscles of facial expression and:
|
| III | Internal carotid artery | Glossopharyngeal nerve (CN IX) | Greater horn of hyoid and inferior hyoid body |
|
| IV | Right subclavian artery aortic arch | Superior laryngeal nerve (CN X) | Laryngeal cartilages | Pharyngeal constrictors, cricothyroid, levator veli palatini |
(A) The head and neck region of a 5-week-old embryo. (B) Horizontal section through the embryo illustrating the relationship of the cervical sinus to the branchial arches and pharyngeal pouches. (C) The child’s neck region, indicating the former sites of openings of the cervical sinus and the pharyngeal pouches. The dotted lines indicate possible courses of branchial fistulas. (D) The embryologic basis of various types of branchial sinuses. (E) A branchial fistula resulting from persistence of parts of the second branchial cleft and the second pharyngeal pouch. (F) Possible sites of branchial cysts and openings of branchial sinuses and fistulas. A branchial vestige is also illustrated.
From Moore KL. The Developing Human: Clinically Oriented Embryology . WB Saunders; 1977.
The presentation of a branchial anomaly can be quite variable. Often, the small, open, fistulous tract or sinus can go unnoticed for months to years. Typically, mucoid or purulent drainage from the aperture just anterior to the sternocleidomastoid muscle prompts parental concern and presentation to the clinic. It is also not uncommon for infection to be the first symptom of a branchial cleft cyst, although this presentation is less common for sinuses or fistulous tracts. The ostium is often accompanied by surrounding skin tags or cartilage remnants. Further history and physical examination findings may include a palpable, subcutaneous cord-like structure or expression of mucoid material through the skin ostium. In addition, the recurrence of swelling or infection may raise suspicion of an underlying branchial anomaly. Children undergoing recurrent incision and drainage procedures should raise the clinician’s suspicion of an underlying branchial anomaly.
The differential diagnosis should include not only congenital benign lesions such as dermoid cysts, lymphatic malformations, or other branchial anomalies, but also infectious processes and malignancy. Often, a more refined diagnosis can be rendered with imaging studies such as computed tomography (CT) and magnetic resonance imaging (MRI). CT has been shown to be capable of identifying a tract in about two-thirds of cases. Ultrasound has been shown to be useful in determining not only cystic versus solid lesions, but also lesions with internal blood flow. However, ultrasound has been shown to have limitations in determining the anatomic extent and involvement of surrounding structures. Upper endoscopy may be required to determine the presence of a pharyngeal opening. A barium esophagram can also be used to identify a pharyngeal tract but only has a 50%–80% sensitivity.
Treatment of congenital branchial anomalies includes complete surgical excision. Surgery can usually be deferred until 3–4 years of age, but some surgeons advocate for earlier excision to avoid recurrent infection ( Fig. 69.2 ). , Indeed, up to 20% of lesions will have been infected at least once prior to definitive surgery. Surgical excision should be avoided in the acute infectious or inflammatory period, and treatment should consist of antibiotic therapy and warm compresses. If drainage is necessary, needle aspiration is preferred to decrease scarring and limit disruption of surgical planes during definitive surgery. The time to wait between infection and definitive surgery is controversial, but most will advocate allowing 4–6 weeks for the infection and inflammation to resolve. ,
(A) At birth, this child was noted to have a right second branchial cartilaginous remnant ( arrow ). However, the family decided not to seek consultation for removal until he was 7 years old. (B) On the right, the cartilaginous remnant has been excised through a small right cervical incision.
First Branchial Cleft Anomalies
The first branchial cleft, arch, and pouch triad form portions of the mandible and contribute to the maxillary process. Abnormal development of the arch can lead to malformations of the palate, external ear, and the ossicular chain; whereas abnormal development of the cleft can contribute to malformations of the middle ear and eustachian tube, the external auditory canal, and the auricle (resulting in microtia or aural atresia). , ,
First branchial anomalies are uncommon and occur in less than 1% of the population, with a slight female preponderance. They occur in two varieties that were described initially by Walter Work in 1972. Work type I lesions contain ectoderm only and are simple duplications of the membranous external auditory canal coursing lateral to the facial nerve. Work type II branchial anomalies are more common than type I, contain both mesoderm and endoderm-derived structures, and course medial to the facial nerve ( Figs. 69.3 and 69.4 ). ,
Type I first branchial cleft anomaly ( FBA ). Note that the anomaly, located in the parotid gland, has no connection to the external auditory canal ( EAC ).
From Mukherji SK, Fatterpekar G, Castillo M, et al. Imaging of congenital anomalies of the branchial apparatus. Neuroimaging Clin N Am . 2000;10:75–93.
Type II first branchial cleft anomaly ( FBA ). The anomaly connects with the external auditory canal ( EAC ) and extends deep into the parotid gland.
From Mukherji SK, Fatterpekar G, Castillo M, et al. Imaging of congenital anomalies of the branchial apparatus. Neuroimaging Clin N Am . 2000;10:75–93.
First branchial anomalies typically appear near the ear lobe but can also appear near the anterior border of the sternocleidomastoid muscle in level 2 of the neck, with external openings inferior to the mandible but superior to the hyoid as well as recurrent preauricular or parotid swelling. About one-third of lesions will open into the external auditory canal and present with recurrent otorrhea. , Surgical excision is difficult as these lesions are often intimately involved with the parotid gland and the facial nerve. This knowledge is important during surgical planning and in counseling the patient and family about the potential risks of surgery. Facial nerve monitoring during surgical excision should be considered as it has been shown to reduce the risk of immediate postoperative facial weakness, although it has no bearing on the risk of permanent facial nerve outcomes.
Surgical resection of first branchial anomalies should include the involved skin or cartilage of the external auditory canal and often requires a superficial parotidectomy with at least a partial facial nerve dissection. Anomalies with tracts that extend to the middle ear place the facial nerve at greater risk during surgical excision as the tract travels superior or inferior to the main trunk of the facial nerve or between the superior and inferior division at the pes anserinus. A planned parotidectomy incision allows for the best exposure, and identification of the facial nerve at the tympanomastoid suture line medial to the posterior belly of the digastric muscle is the most reliable intraoperative landmark. The risk of temporary facial nerve injury approaches 30%, and permanent facial nerve paralysis occurs 12% of the time when the facial nerve is not identified and dissected during the procedure. Postoperative recurrence is not uncommon and multiple procedures may be required, with each subsequent procedure placing the facial nerve at greater risk.
Second Branchial Cleft Anomalies
Second branchial cleft anomalies are the most common, representing about 95% of all branchial anomalies, and are usually found along the anterior border of the sternocleidomastoid muscle. The second arch consists of Reichert’s cartilage that gives rise to the styloid process, the manubrium of the malleus, the long process of the incus, the stapes suprastructure, the stylohyoid ligament, the superior hyoid body, and the lesser cornu of the hyoid. , Its arterial structures include the stapedial artery and the caroticotympanic artery. The facial nerve is the neural structure associated with the second branchial arch, and its muscles are those innervated by cranial nerve VII including the muscles of facial expression, the posterior belly of the digastric, the stylohyoid, and the stapedius. , , Since the second cleft tract penetrates the platysma and all layers of cervical fascia, remnants can be found anywhere along a course from the skin, to or through the carotid sheath, and ending at the nasopharynx or oropharynx. Classically, the tract passes between the internal and external carotid, posterior to the posterior belly of the digastric and stylohyoid muscles, and over the hypoglossal nerve to terminate in the tonsillar fossa. However, the course and presentation can be quite variable and are grouped into four types. In type I lesions, the cyst is superficial to the sternocleidomastoid muscle. Type II lesions are the most common and are located deep to the sternocleidomastoid muscle before coming to rest adjacent to the carotid sheath. Type III lesions are as described classically above, passing between the branches of the carotid and ending adjacent to the pharynx. Type IV is deep to the carotid sheath abutting the pharynx ( Fig. 69.5 ).
Types I–IV second branchial cleft anomalies. (A) Type I: the cyst (C) is superficial to the sternocleidomastoid muscle ( M ). (B) Type II: the cyst is adjacent to the carotid sheath. (C) Type III: the cyst passes between the internal and external carotid arteries to the lateral wall of the pharynx ( P ). (D) Type IV: the cyst is deep to the carotid sheath abutting the pharynx.
From Mukherji SK, Fatterpekar G, Castillo M, et al. Imaging of congenital anomalies of the branchial apparatus. Neuroimaging Clin N Am . 2000;10:75–93.
Surgical excision of second branchial cleft anomalies should follow the course of the fistula or cyst, taking care to avoid iatrogenic injury to the major vessels as well as the spinal accessory, vagus, and hypoglossal nerves. A stairstep incision is often required for better exposure as the tract approaches the pharynx ( Fig. 69.6 ). Care should be taken to adequately ligate the tract at its entry point to the pharynx as inadequate control may lead to a salivary fistula and recurrent infection. Several surgical alternatives exist for treatment, including a guidewire stripping technique, retroauricular hair line approach for cosmesis, and endoscopic approaches. All of these are limited by poor long-term data and procedural learning curves. Several studies have also suggested that sclerotherapy with either OK-432 or ethanol can result in improvement or resolution of these lesions with acceptable cosmesis.
This 14-year-old male had a left-sided draining fistulous tract since birth that was shown to be a Type II branchial cleft fistula extending to the level of the oropharynx. A stair-step incision was required for safe exposure during excision.
Third and Fourth Cleft Anomalies
Third and fourth branchial anomalies are rare. In general, the third and fourth arches form the pharynx below the level of the hyoid bone and therefore fistulas enter the hypopharynx. The third pharyngeal arch gives rise to the inferior body and greater cornu of the hyoid bone as well as the stylopharyngeus muscle. Its neurovascular structures include the common and internal carotid arteries as well as the glossopharyngeal nerve. The third pharyngeal pouch gives rise to the inferior parathyroid glands and the thymus. The fourth pharyngeal arch gives rise to the thyroid cartilage, cuneiform cartilages of the larynx, and the epiglottis in addition to the pharyngeal constrictors, cricothyroid muscles, and the levator veli palatini. Its neurovascular structures include the aortic arch and the right subclavian artery as well as the superior laryngeal branch of the vagus nerve. , , This structure also gives rise to the superior parathyroid glands as well as the ultimobranchial body, to the origin of the parafollicular cells in the thyroid gland.
Presentation of third and fourth branchial anomalies is similar, and most authors will group them together owing to their similar treatment strategies. Fourth branchial anomalies are usually found on the left side of the neck. Often, differentiation is noted only intraoperatively, as third branchial anomalies will be superior to the superior laryngeal nerve and fourth branchial anomalies inferior to the superior laryngeal nerve. Cysts and sinuses often present lower in the neck than second branchial anomalies. Their tracts will travel deep to the carotid sheath between the glossopharyngeal and hypoglossal nerves before terminating in the pyriform sinus. Indeed, direct visualization of a tract in the pyriform sinus on endoscopy, barium esophagram, or CT is diagnostic. Owing to their anatomic course, rapid enlargement of a third or fourth branchial anomaly may lead to tracheal compression, but recurrent infection remains the most common presentation. About one-third of patients with a third branchial anomaly and about 40% of patients with a fourth branchial anomaly will present with either a neck abscess abutting the thyroid gland or suppurative thyroiditis. Due to their relative rarity, other cystic lesions should be entertained in the differential diagnosis. Thymic cysts can result from incomplete degeneration of thymalpharyngeal duct or from progressive cystic degeneration of Hassall corpuscles. Parathyroid cysts can also be seen near the thyroid gland or mediastinum. Their cause is not entirely clear but may be a cystic remnant of a third or fourth branchial apparatus, cystic degeneration of an adenoma, or enlargement of microcysts. Patients are typically euparathyroid. Finally, fourth branchial anomalies can also mimic laryngoceles as their tracts pierce the thyrohyoid membrane en route to the pyriform sinus ( Figs. 69.7 and 69.8 ). ,
Third branchial cleft anomaly. The cyst ( C ) is posterior to the sternocleidomastoid muscle, and the tract ascends posterior to the internal carotid artery. It then passes medially between the hypoglossal ( H ) and glossopharyngeal ( G ) nerves. It pierces the thyroid membrane ( M ) to enter the pyriform sinus.
From Mukherji SK, Fatterpekar G, Castillo M, et al. Imaging of congenital anomalies of the branchial apparatus. Neuroimaging Clin N Am . 2000;10:75–93.
Fourth branchial cleft anomaly. The cysts ( C ) are located anterior to the aortic arch on either side. The tract hooks either the subclavian artery or the aortic arch, depending on the side, and ascends to loop over the hypoglossal nerve ( H ).
From Mukherji SK, Fatterpekar G, Castillo M, et al. Imaging of congenital anomalies of the branchial apparatus. Neuroimaging Clin N Am . 2000;10:75–93.
Classically, as with other branchial anomalies, complete surgical excision is the gold standard treatment of choice with a few notable intricacies. Many surgeons will find it fruitful to perform endoscopy at the beginning of the case and cannulate the pyriform sinus tract to aid in surgical dissection. Often, hemithyroidectomy is needed for complete excision, particularly for fourth branchial anomalies, and occasionally partial resection of the thyroid cartilage is necessary to trace the tract to its origin in the pyriform sinus. Some authors have also suggested that surgical excision be delayed until children are 8 years of age or older due to a decreased risk of postoperative complications, but if a child is having recurrent infections, a risk-benefit analysis may dictate earlier surgical intervention. Endoscopic tract cauterization has gained popularity as an alternative to complete excision for its efficacy and safety with success rates after a first and second cauterization of between 66.7%–100% and 77.8%–100%, respectively. The patient should be intubated for this procedure, and a laryngoscope is used to expose the tract in the pyriform sinus. A blunt probe is then used to dilate the tract followed by insertion of a Bugbeeä monopolar electrocautery device (Medline Industries). The tract is then cauterized on a low setting as it is slowly withdrawn to denude the tract epithelium, allowing for scar formation and tract involution ( Fig. 69.9 ). This approach is less invasive, avoids open surgical complications, and decreases hospital length of stay. , Pitfalls of this procedure can include a temporary vocal fold paresis secondary to thermal spread of the electrocautery device, irritation of the recurrent laryngeal nerve, as well as temporary dysphagia due to irritation of branches of the superior laryngeal nerve from the same mechanism. If this approach fails, open excision is recommended.
This 9-year-old female presented with left-sided suppurative thyroiditis suggestive of a third branchial anomaly. Six weeks following completion of antibiotic treatment, she underwent endoscopic tract identification in the left pyriform sinus (A) and tract obliteration with a Bugbeeä cautery (B).
Preauricular Cysts and Sinuses
The auricle and external ear develop from the first and second branchial arches. By the fifth week of gestation, six individual small buds of mesenchymal tissue arise around these arches. Thus, preauricular pits, cysts, and sinuses are not of true branchial cleft origin, but rather represent ectodermal rests as they relate to the auditory hillocks (hillocks of His). The hillocks fuse by the end of the 12th week of gestation to form the complete auricle. Failure of the epithelium of the first and second hillock to resorb leads to a cyst or sinus formation. Due to this embryological relationship, preauricular sinuses are often short and end blindly. They do not connect to the external auditory canal or tract through the parotid gland. Thus, the facial nerve is not at-risk during resection and remains deep to an uninterrupted parotid fascia. The tract for preauricular sinuses will blend with the perichondrium of the helical crus; therefore, many authors will advocate for removal of a small piece of the helical cartilage along with the sinus or cyst. ,
Preauricular pits, cysts, and sinuses are relatively common, with an incidence of 0.1%–0.9% in the United States and up to 4%–10% in Africa. These lesions occur anterior to the root of the helix and have a slight right-sided predominance. Typically, the lesion is asymptomatic and without any associated findings. Occasionally, there is a hereditary component, particularly in families with branchio-oto-renal syndrome. Patients with preauricular pits and an associated finding such as hearing loss should undergo genetic testing and a renal ultrasound, but the presence of preauricular pits or tags alone has not been shown to be different from the general population.
The diagnosis is clinical, but occasionally ultrasonography, CT, or MRI may be needed to narrow the differential diagnosis if the clinical picture is not clear. The greatest risk is recurrent local infection, and many authors will advocate for prophylactic surgical intervention; however, most of these lesions will not become infected. Preauricular sinuses that drain do have a greater risk of recurrent staphylococcal infection or chronic staphylococcal colonization. Early excision in these cases is paramount as continued infection increases the difficulty of surgery and therefore increases recurrence ( Fig. 69.10 ).
An infected preauricular cyst. The pit anterior to the helix is difficult to see. Note the swelling and skin changes anterior to the tragus. Preauricular sinuses that drain sebaceous material should be excised electively. Warm compresses and antibiotics allowed the inflammation to diminish. The cyst and sinus were then completely excised.
Complete excision of the sinus tract and its cystic components down to the level of the temporalis fascia is the treatment of choice. Avoiding rupture of the cyst is important in preventing recurrence. If the cyst is acutely infected, treatment with needle aspiration as opposed to incision and drainage, has been shown to reduce the rate of recurrence. Excision is accomplished via a small, elliptical incision around the punctum of the sinus. The underlying cyst is then freed from the deeper fascia and removed in its entirety. A lacrimal probe can be used to help determine the direction and the depth of the tract if it is not clinically apparent. Since the cyst often has multiple small branches, taking a small amount of perichondrium or helical cartilage will reduce the risk of recurrence. Recurrence rates can be as high as 42%, and when recurrence does occur, wide, local excision with full thickness excision of the adjacent helical cartilage may be necessary. Extending the incision superior around the helical root has also been shown to reduce rates of recurrence as it facilitates exposure of the helical root and underlying temporalis fascia. Rotational flaps may be necessary to cover large defects, but local advancement flaps are usually sufficient.
Thyroglossal Duct Cysts
Thyroglossal duct cysts are the second most common pediatric neck mass behind only cervical lymphadenopathy. Remnants of the thyroglossal tract are found in about 7% of the population. , Despite their congenital nature, they are not typically seen in the perinatal period or in infants and usually do not present until children have reached age 3–5. Two-thirds of thyroglossal duct anomalies are diagnosed within the first three decades of life.
The thyroid gland begins its descent into the neck from the foramen cecum at around 4 weeks of gestation. The thyroglossal tract then passes through the tongue before descending anterior, posterior, or, most commonly, through the developing hyoid bone prior to reaching its final pretracheal position by week seven of gestation. The thyroglossal tract should then involute and disappear by week 6–8 of gestation. , Given the developmental path of the lesion, a thyroglossal duct cyst may develop anywhere along the tract from the base of the tongue to the lower neck, with the most common location being adjacent to the hyoid bone. , It is important to note that thyroglossal duct remnants will never have an external opening due to their embryonic origins ( Fig. 69.11 ).
Thyroglossal duct cysts can be located anywhere from the base of the tongue to behind the sternum. (A and B) Lingual (rare); (C and D) Adjacent to hyoid bone (common); (E and F) Suprasternal fossa (rare).
From Welch KJ, Randolph JG, Ravitch MM, et al., eds. Pediatric Surgery . 4th ed. Year Book Medical; 1986:549.
The initial presentation is that of a painless midline neck mass with two-thirds found adjacent to the hyoid bone. Suprahyoid anomalies need to be differentiated from submental lymphadenopathy or a dermoid cyst. , Thyroglossal duct cysts are smooth, soft, and nontender cysts that ascend in the neck with tongue protrusion or deglutition ( Fig. 69.12A ). Mobility with tongue protrusion has been shown to be a more reliable physical exam finding. Communication of the thyroglossal duct tract with the foramen cecum and therefore the oral cavity means primary infection of a thyroglossal duct cyst is not uncommon and can be the herald sign for up to one-third of lesions. Of lesions that present with infection, 25% will present with a draining sinus from a spontaneously draining abscess or require incision and drainage ( Fig. 69.12B ). Rarely, spontaneous drainage occurs into the oral cavity, resulting in a complaint of a poor taste in the mouth or halitosis.
These two children have thyroglossal duct cysts. (A) On the left , the cyst is noted on this lateral photograph. There is no evidence of prior infection. (B) On the right , the area developed an infection and required incision and drainage prior to operative excision.
The diagnosis is primarily clinical. Thyroid function testing is not indicated unless the patient has clinical signs and symptoms of hypothyroidism. A normal thyroid gland is nearly always present as the thyroglossal duct cyst is due to failure of tract involution following thyroid formation. Ultrasonography is the imaging modality of choice as it avoids radiation exposure and can be done without sedation, but it has been shown to have difficulty in differentiating thyroglossal duct cysts from midline neck masses. However, ultrasonography can be useful in determining cystic from solid lesions such as ectopic thyroid tissue.
Ectopic thyroid tissue can be found in up to 45% of pathologic specimens. , Therefore, some authors suggest either thyroid scanning or ultrasound to determine its presence and to differentiate it from a thyroglossal duct cyst. , Up to 90% of ectopic thyroid tissue forms at the base of the tongue, a rare location to find a thyroglossal duct cyst. Ectopic thyroid tissue occurs in 1:100,000 individuals, with a female preponderance of 4:1. Nearly 33% of people with ectopic or lingual thyroid will be hypothyroid, so thyroid function testing is recommended in patients with clinical lingual or ectopic thyroid tissue, especially when a normal-appearing thyroid in the neck is absent. Indeed, 70%–100% of individuals with ectopic thyroid tissue have no other thyroid tissue in the neck. , CT or MRI may be useful to better delineate the surrounding anatomy. Due to the location and a lack of other thyroid tissue in the neck, a trial of thyroid suppression is often performed and occasionally treatment with I 131 to medically debulk the lesion.
Thyroglossal duct carcinoma occurs in fewer than 1% of cases, with papillary and follicular being the most common. Most of these carcinomas are discovered only during pathologic examination of the surgical specimen. Once this pathologic diagnosis is made, I 131 scanning is recommended to ensure the remaining thyroid gland is present and to rule out other nodules in the gland. Further surgery is contraindicated if the scan is normal. If further disease is suspected, a total thyroidectomy is indicated. Further discussion on pediatric thyroid carcinoma is beyond the scope of this chapter.
Elective surgical excision is the current recommended treatment using the technique described by Schlange in 1893 and modified by Sistrunk in 1920. , During this procedure, the cystic lesion is excised along with a wide tract of tissue, the midportion of the hyoid bone, and a core of base of tongue musculature, creating a pharyngotomy. Currently, most surgeons perform a modified version of this procedure, in which a core of the base of tongue musculature is excised without creating a pharyngotomy. Wide excision is necessary as several studies have suggested that several small tracts can emanate from the primary tract, which occur primarily cephalad to the hyoid bone. If wide excision is not employed to excise these tracts, the rate of recurrence increases from 1.9%–6.5% to 23%–50%.
Congruent with most neck surgeries, the patient should be laid supine on the operating room table with a shoulder roll to gently extend the neck. Perioperative antibiotic use is controversial, but selection of antibiotic therapy should include agents covering both Staphylococcal species and oral flora, typically ampicillin-sulbactam. The thyroglossal duct cyst is then exposed through a transverse midline neck incision directly over, or just inferior, to the cyst itself. The platysma is often diastatic in the midline and may not be encountered. Further deep dissection is carried through the superficial layer of the deep cervical facia until the strap muscles are identified. These are then separated in the midline and retracted laterally. If the thyroglossal duct cyst is large enough, the strap muscles may already be lateralized, and great care must be taken to not violate the cyst. The thyroglossal duct cyst has a characteristic appearance that is distinct from the surrounding muscle and laryngeal cartilage. Dissection then proceeds around the cyst including a block of tissue along its tract proceeding cephalad to the level of the hyoid bone. Once the hyoid bone is encountered, development of a posthyoid space is undertaken, and the midportion of the hyoid bone is transected with heavy Mayo scissors, a rongeur, or Pott’s scissors. In younger patients, the hyoid bone is rather thin and malleable and can be divided with electrocautery. Some authors advocate for the use of ultrasonic osteotomy devices to minimize damage to surrounding structures, but this method has not been shown to have a significant effect on complication rates. Placing a finger into the oropharynx and applying pressure will help identify the cephalad-most point of dissection. The base of the tract at the tongue base is then ligated with absorbable suture and/or heavily cauterized to ablate any remaining microscopic suprahyoid tract. If a pharyngotomy is intentionally or unintentionally created, it should also be closed with absorbable sutures. The edges of the hyoid bone do not need to be reapproximated, but if there is ongoing oozing from the cut edges, bone wax may be used to help with hemostasis. After copious irrigation with normal saline, the wound is then closed in layers. Drain placement is also controversial as no difference has been found in rates of postoperative complications in patients with a drain placed versus those without.
Indications for excision of ectopic thyroid tissue include dysphagia, dysphonia, hemorrhage, respiratory distress, and concern for malignancy. Surgical approaches include transhyoid pharyngotomy, lateral pharyngotomy, midline labiomandibular glossotomy, and transoral excision. Most specimens of ectopic or lingual thyroids reveal histologically normal thyroid tissue.
Nonsurgical management of thyroglossal duct cysts has gained popularity over the last decade, particularly for patients who do not wish to have a scar on their neck. Positive results for cyst volume reduction, recurrence, and cosmesis have been well described for adult patients. However, thyroglossal duct cyst sclerotherapy in children has remained largely poorly described with limited longitudinal data. The need for further surgical intervention has been shown to be about 10%–20%. , Ablation with OK-432 has not been shown to influence the complexity or complication rate of subsequent salvage surgery.
Major and minor complications can be encountered during or following excision of thyroglossal duct cysts. Major intraoperative complications include tracheotomy or cricothyrotomy, hypoglossal or recurrent laryngeal nerve injury, or death. Major postoperative complications include recurrence, hematoma, abscess, and hypothyroidism. Minor complications can include seroma formation, wound dehiscence, local wound infection, or stitch abscess; minor complications occur in up to 30% of patients.
Of the major complications, cyst recurrence is by far the most common. Risk factors include simple cyst excision alone with recurrence rates between 38% and 70%, intraoperative cyst rupture, the presence of a cutaneous component due to infection, and postoperative wound infections. , The risk of recurrence has been shown to be reduced in several series by performing extensive excision of the infrahyoid tissue with exposure of the posthyoid space, noting that arborization of the thyroglossal duct tract can exist along its length and not simply at the base of the tongue or foramen cecum. This approach to primary surgery has shown a recurrence rate between 0% and 1.67%. , During revision surgery, the limits of the primary excision versus scarring are often blurred. Several authors advocate for block dissection of all nonvital structures of the central neck between the digastric muscles and over the strap muscles, including further dissection of the suprahyoid tissue and any abnormal skin.
Whether preoperative or postoperative, infected cysts, sinuses, or wound beds should be managed by treating the infection with directed antibiotics covering Staphylococcal , Haemophilus , and Streptococcal species. Case reports of postoperative severe infections including sepsis and necrotizing fasciitis have been reported. It is imperative to avoid incision and drainage of an acutely infected cyst, but, if necessary, the incision should be placed in a location where the cyst can be later excised during definitive surgery. Attempted excision of an acutely infected cyst carries a recurrence rate of up to 25%. Some authors advocate for waiting 3 months or more for inflammation to resolve prior to excision.
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