utero atrial septoplasty in fetuses with hypoplastic left heart syndrome. Of 21 procedures attempted, 19

were technically successful. Although the authors could not conclude that this particular procedure

improved survival given the small patient size, they did find that creation of a defect greater than 3 mm

was associated with better postnatal oxygenation and less frequent need for emergent postnatal

intervention.111

Fetal Intervention for Other Anomalies

Fetal intervention is indicated for a number of other fetal conditions, including amniotic band syndrome

and twin-reversed arterial perfusion (TRAP) sequence. Fetoscopic lysis of constricting amniotic bands

may lead to limb salvage and improved outcome.115–117 Similarly, in the TRAP sequence, fetoscopic

ligation or ablation of the acardiac twin’s umbilical cord is the treatment of choice.118

FUTURE OF FETAL INTERVENTION

There will always be a role for fetal intervention in the treatment of certain specific congenital

structural abnormalities. The exact nature of this role, however, is under constant reevaluation and

change. The primary goal of fetal therapy is to improve the outcome of the affected fetus while

minimizing maternal risks. If fetal outcomes can be improved by alternative means, such as lung liquid

ventilation or other postnatal therapies that pose no risks to the mother, then indications for fetal

therapy will decrease. On the other hand, as the risks and complications of fetal surgery continue to

decrease, such as those related to preterm labor and chorioamniotic membrane separation, then the role

for fetal interventions may increase. Increased miniaturization of fetoscopic equipment and optics,

advances in robotics, newer approaches to closure of the uterus and fetal membranes, and advances in

our understanding and treatment of preterm labor will enhance our ability to change the outlook for an

increased number of fetuses with congenital malformations.

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Chapter 101

Pediatric Head and Neck

Laura L. Neff and Reza Rahbar

Key Points

1 Infants are obligate nasal breathers for the first 3 to 6 months of life; therefore, any nasal

obstruction can cause respiratory distress.

2 The site of the neck mass is important (midline vs. lateral) for narrowing the differential diagnoses.

3 Benign cervical lymphadenopathy is the most common neck mass in the pediatric population.

4 Although rare, always consider malignancy to avoid delay in diagnosis.

5 History is important in the work up and treatment of an airway foreign body.

INTRODUCTION

Lesions of the head and neck in the pediatric population result from a wide range of etiologies. In

contrast to the adult population, the majority of pediatric neck masses are benign with only 11% of

neck masses demonstrating a malignancy on biopsy.1 In pediatrics, neck masses can be congenital,

infectious, inflammatory, traumatic, lymphovascular, or neoplastic in etiology. Neck masses can range

in presentation from an asymptomatic lesion to acute respiratory distress, depending on their size and

location. Inflammatory cervical lymphadenitis is the most common cause of pediatric neck masses found

in children. Congenital masses, such branchial cleft cysts and thyroglossal duct cysts (TGDC), often

become clinically apparent when infected. Malignant processes are rare in children, and the most

common malignancy seen in the pediatric neck is lymphoma. This chapter will focus on the most

common head and neck pathologies found in the pediatric population along with their clinical

presentation and treatment.

Imaging

Imaging is not always required for the diagnosis of a pediatric neck mass, but can be helpful in

clarifying extent and size, location to vital structures, and radiologic characteristics of a lesion.

Ultrasound (US), computed tomography (CT), or magnetic resonance imaging (MRI) can be helpful in

narrowing the differential, but often a biopsy or excision is still required for diagnosis.

US is a good first choice for imaging of palpable lesions such as salivary gland tumors or thyroid

nodules. The pediatric neck often has less subcutaneous adipose tissue, which leads to better imaging

with ultrasonography.2 US offers the ability to differentiate solid from cystic lesions, and color Doppler

can determine the presence and characteristics of vascular flow within a mass.3 US can be very helpful

in determining the stage of an abscess, helping to differentiate early lymphadenopathy that can be

treated with antibiotic therapy from a mature abscess that requires surgical drainage. US has the

advantages of being portable, fast, does not require sedation, and does not expose the pediatric patient

to ionizing radiation. Disadvantages of US include variability of the technician’s skill/experience as well

as lower resolution images.

CT scans and MRI provide a more detailed study of the anatomy of the neck. CT scans are readily

available in emergent situations with scans completed quickly for patients who are critically ill. This

speed can also help to eliminate the need for sedation and help to minimize artifact from movement. CT

scans offering more osseous detail such as remodeling or erosion, as well as calcifications. The main

disadvantage of CT scans is the exposure to ionizing radiation, which could potentially have

carcinogenic effects in the long term.4–6 Newer techniques offer reduced exposure with CT scans, but

MRI and US should be utilized when appropriate.

MRI is the scan of choice for neck masses due to excellent soft tissue detail and avoidance of ionizing

radiation. Details of the margins and possible neural spread or intracranial extension of a mass can be

noted with the use of a contrast-enhanced MRI. The longer length of time required to obtain an MRI

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often requires sedation due to concerns of movement artifact in the pediatric population, which is the

main disadvantage of the MRI.

To help with evaluating, staging, and monitoring a solid tumor, 18F fluorodeoxyglucose PET (FDGPET) is a noninvasive tool to evaluate malignancies in children. FDG-PET has the advantage of being

able to differentiate recurrent or residual tumor from changes related to treatment. PET scans do expose

children to ionizing radiation and also may require sedation. It is also important to keep in mind that

certain tissues have a higher uptake of FDG, including the adenoids, tonsils, thymus, brown adipose

tissue, bone marrow, and the spleen.7,8

Congenital

Congenital lesions of the head and neck are diverse and often the location of the lesion can greatly

assist in narrowing the diagnosis. For example, midline lesions include TGDCs and der-moid cysts,

whereas lateral neck lesions have a much broader differential.

Branchial Anomalies

The branchial apparatus in the fetus eventually develops into head and neck structures. Any deviation in

the development of these structures can result in an anomaly that can present as a mass in the neck.

Depending on the part of the branchial apparatus that is involved, the mass can present in different

parts of the neck. Sinus tracts and fistulas tend to present at a younger age due to their physical

appearance, whereas cysts usually present when they get infected and enlarge. A sinus tract has an

external opening to the neck, whereas a fistula has an external and internal opening. Cartilaginous

remnants can also be found in the neck.

Figure 101-1. CT or MRI of second branchial cleft cysts.

These lesions often present with infection, and it is best to minimize inflammation and infection with

antibiotics prior to excision, making the dissection and identification of important structures easier.

Abscesses can be treated with minimal intervention, such as needle aspiration or if needed, incision and

drainage. During the procedure, it is important to communicate with anesthesia to avoid paralysis for

proper nerve monitoring. Radiologic imaging with CT or MRI can be helpful (Fig. 101-1). Rates of

recurrence for branchial cleft cysts is quoted around 3%, but this increases to 20% in cases that have

undergone a prior attempt at excision.9

Anatomy and Embryology

The branchial arches form during the fourth to eighth week of gestation as four pairs of well-developed

ridges with associated clefts. Each has a cartilaginous center (mesoderm), a cleft (ectoderm), an internal

pouch (endoderm), and a nerve (Table 101-1). These structures mature into the major structures of the

head and neck (Fig. 101-2).

Table 101-1 Pharyngeal Arch Structures

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Figure 101-2. Derivation of various areas of the head and neck from the branchial arches and clefts of the embryo.

First branchial cleft anomalies, which comprise 8% of branchial cleft anomalies, are divided into type

1 and type 2.10 Type 1 branchial cysts often present as a fistula around the conchal cartilage. The tract

can follow the course of the external auditory canal and is lined by squamous epithelium.11 Type 2

branchial lesions are found near the angle of the mandible and are composed of both epithelium and

mesoderm (as opposed to only epithelial elements in type 1). Both type 1 and type 2 first branchial cleft

anomalies can be closely associated with the facial nerve, although type 2 lesions are more likely to be

intimately involved with the nerve. Type 2 branchial lesions can loop under the facial nerve, pushing it

laterally and inferiorly.12 The tract for a first branchial cleft usually extends from the opening near the

mandible, near the posterior aspect of the parotid, and toward the external auditory canal. One should

monitor the facial nerve at all times, with visualization of the face during the entire excision and

avoidance of paralysis with anesthesia.

The most common branchial anomaly is derived from the second branchial cleft groove, representing

90% of branchial cleft anomalies.13 It can occur as an isolated cyst or as a sinus tract/fistula that extends

from the cervical skin to the tonsillar fossa. The cysts will often present at the time of an upper

respiratory tract infection as an enlarging, tender mass. If a fistula is present, it will course through the

internal and external carotid arteries, over the hypoglossal and glossopharyngeal nerves to end in the

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tonsillar fossa. A cyst can occur anywhere along this tract, but most commonly occurs in the anterior

triangle of the neck below the hyoid.

Third and fourth branchial cleft lesions are rare. Third branchial cleft cysts present low and anterior

in the neck. Fistulas can course from the piriform fossa and drain to the anterior cervical skin. This tract

pierces the thyrohyoid membrane and tracks under the glossopharyngeal nerve and internal carotid

artery, but stays above the vagus nerve. These lesions can be intimately involved with the thyroid, and

sometimes a hemithyroidectomy is required for repeated recurrences. An infected TGDC or third

branchial cleft cyst may be the cause of suppurative thyroiditis in children.14 The external location for a

fourth branchial cleft is the same as the second and third, but the internal opening is near the apex of

the piriform sinus. Fourth arch masses are extremely rare and but reports do exist. Third and fourth

branchial sinus/tract dissections are similar to a second branchial cleft. Endoscopy at the beginning of

the case can greatly assist in locating and resecting the tract. One promising surgical option for third or

fourth sinus tracts is cauterization or sclerotherapy.15

The standard treatment for all types of branchial anomalies is complete surgical excision. The surgery

is performed under general anesthesia and the patient is positioned with the neck slightly extended with

a shoulder roll. If there is a sinus tract or fistula present, a small ellipse is made around this opening

after it has been cannulated with a small lacrimal probe. A probe can be used or some use a small

injection of methylene blue, although this can be messy. The tract is then dissected cephalad until the

end of the sinus tract or until the internal opening of the fistula is reached. The dissection for the most

common branchial cleft anomaly, the second branchial cleft, penetrates the platysma, rises along the

carotid sheath and turns medially near the branches of the internal carotid artery and courses between

the posterior belly of the digastric muscle and stylohyoid muscle, and over the hypoglossal nerve before

ending near or in the tonsillar fossa. The tract is then ligated with absorbable suture. Often a direct

laryngoscopy or simply a finger placed in the oropharynx can help to visualize the course of the tract

and assist with dissection. Some patients might require a “stepladder” incision to trace the tract

superiorly (Fig. 101-3).

Cartilaginous remnants are normally small and present in the subcutaneous tissue along the anterior

border of the sternocleidomastoid, are usually palpable and easily resected. These rarely get infected

and removal is for cosmetic indications.

Preauricular cysts, pits, and sinuses are common in children and are thought to develop from the first

two branchial arches. They are more common and more often bilateral in comparison to first branchial

cleft cysts and rarely become infected. The tracts extend from the skin down to the helical cartilage of

the auricle and are lined with squamous epithelium. Treatment is surgical excision and the recurrence

rate ranges from 19% to 40%.16

Figure 101-3. Picture of sinus tract.

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