Editorial
Pediatric Congenital Neck Masses: A Review Article
Erikci VS*
Department of Pediatric Surgery, Sağlık Bilimleri University, Tepecik Training Hospital, Turkey
*Corresponding author: Volkan Sarper Erikci, Department of Pediatric Surgery, Sağlık Bilimleri University, Tepecik Training Hospital, Kazım Dirik Mah. Mustafa Kemal Cad. Hakkıbey apt. No:45 D.10 35100 Bornova-İzmir, Turkey
Published: 01 Dec, 2017
Cite this article as: Erikci VS. Pediatric Congenital Neck
Masses: A Review Article. Clin Surg.
2017; 2: 1791.
Keywords
Congenital neck mass; Thyroglossal duct remnant; Branchial cleft anomaly; Dermoid cyst
Editorial
Congenital neck lesions are common clinical concern in infants and children. The differential
diagnosis includes congenital, inflammatory and neoplastic lesions. The physicians caring for
children with Congenital Neck Lesions (CNLs) should be aware of different presentations since
these lesions are known to be complicated by infection. An orderly examination of the neck with a
clear understanding of embryology and anatomy of the region will facilitate the diagnosis. Surgical
intervention is cornerstone in the treatment and early referral of these patients for pediatric surgeons
is recommended. The most common CNLs encountered in pediatric practice are Thyroglossal Duct
Remnants (TGDR) followed by Branchial Cleft Anomalies (BCA) and Dermoid Cysts (DC) [1].
With a correct preoperative diagnosis and appropriate surgical management it is possible to prevent
recurrence that is the most common complication in these lesions. CNLs in children constitute one
of the most intriguing areas of pediatric pathology and can produce diagnostic and therapeutic
challenges for clinicians and surgeons. In the absence of infection, identification of a cystic mass in
a child should raise the diagnosis of congenital malformations related to abnormal embryogenesis
of the thyroglossal duct. TGDRs are the most common form of CNLs, accounting for up to 70-90%
of such lesions [2-4]. In a recent report, more than half of the patients (53.4%) with CNLs presented
with TGDR [5]. The true incidence is not known with certainty and in fact many are never detected
clinically. A 7% incidence of TGDR in a postmortem study of 200 adults has been reported [6].
These lesions are commonly observed in children or adolescents and in a meta-analysis its incidence
was found to be higher in children than in adults [7]. Although there are conflicting reports with
regard to sex distribution [8-10], equal distribution among males and females has been reported in
most of the reviews [7,11,12]. It should be kept in mind that a true female dominance does exist
amongst familial TGDRs [13]. Main presentations of TGDR are that of a midline neck mass or
infection as a single or a recurrent event (Figure 1). Diagnostic methods in the preoperative
evaluation of TGDR include US, CT, MRI, Fine Needle Aspiration (FNA), radioisotope thyroid
scanning, and thyroid function test [14]. US is the most common test ordered in children [15]. US is noninvasive and offers valuable information of both the TGDR and thyroid gland [16]. The
absence of a normal appearing thyroid gland in the lower neck should alert the clinician that ectopic
thyroid tissue might be present within the TGDR or elsewhere along the course of the thyroglossal
duct and thyroid scintigraphy with thyroid function tests should be performed [17]. In the diagnostic
work up, thoracic CT or MRI may be performed for the documentation of other comorbidities as
such when the lesion is extensive crossing multiple anatomical spaces. Concerning FNA, although
the diagnostic sensitivity of 62% and a positive predictive value of 69% is reported, it is not popular
for diagnosing TGDR in children [14,18]. The most common location for the cystic mass in TGDR
is close proximity to the hyoid bone with an incidence of 66%, but other locations including lingual,
suprahyoid, suprasternal or within the thyroid gland have also been reported [7,19]. Although the
incidence of complications including recurrence following the Sistrunk’s procedure has been noted
up to 29% [20], recurrence rate of TGDR after the procedure is reported to be 2.6% to 5% in most
recent literature [21,22]. Deeper and wider excisions including removal of midportion of hyoid
bone in patients without bone resection during the initial operation are suggested to remove any
missed epithelial remnants during the subsequent operations in these patients with recurrence.
Apart from recurrence, possible major complications of TGDR surgery such as abscess or hematoma
requiring surgical drainage, inadvertent entry into the airway, tracheotomy, nerve paralysis,
hypothyroidism are rare [23]. It is reported that fewer than 1% of patients with TGDRs may have
malignant tissue, usually well differentiated thyroid carcinoma [24]. Minor complications may
include seroma, local wound infection and dehiscence [20]. Although the Sistrunk’s procedure is
safe and successful technique with low complication rates, rare and life threatening complications should be kept in mind during the management of these children.
BCAs are the second most common CNL and comprise 20%-30% of
all head and neck lesions [21,25-27]. It is reported that there is an
equal sex distribution in BCAs [25,28]. Although branchial remnants
are relatively rare, which can present in a variety of ways depending
on the origin of the cleft, the most common type is the second cleft
anomalies accounting for 95% of all lesions [1,5]. In classic cases,
complete history and physical examination is adequate for diagnosis
and no additional evaluation may be necessary (Figure 2). An upper
airway endoscopy may be useful in determining the presence of a
pharyngeal opening [21]. On the contrary to adults, FNA should not
be performed and incisional biopsy should be avoided in children
otherwise resection of branchial lesion will be technically more
difficult [25]. US and CT may be helpful in defining the lesion and its
anatomic course (Figure 3). These imaging modalities should be
performed preoperatively for complete visualization of the tract [25].
Common current practice is to obtain a preoperative US for planning
the treatment. BCAs present as cysts, sinuses, or fistulae and clinical
presentation heavily depends on the type of the lesion. Cysts present
as swelling while sinuses or fistulae may produce clear discharge. In a
recent study of the patients, 64% had discharging sinuses or fistulae
and blind ending sinuses outnumbered other presentation types of
BCA [5]. This finding is in accordance with the literature in that
branchial fistulae and sinuses are diseases of childhood while cysts are
more common in adults [29]. In the largest review of BCAs comprising
232 procedures, 90% of which included second branchial anomalies
with an incidence of 13.5%, only 28 children with second branchial
anomalies demonstrated complete fistulae [30]. Although studies of
less invasive procedures are promising including sclerotherapy and
endoscopic excision or cauterization, the definitive treatment of these
lesions has historically been complete surgical excision of the entire
tract [1,31]. There is controversy on the timing of the resection. Some
suggest early resection in order to prevent infection whereas others
advocate waiting until the ages of 2-3 years [25,32,33]. If there is
prolonged period of symptoms before diagnosis and treatment,
timing of surgical intervention may be during late period of childhood.
Recurrence rates following surgical excision have been reported as
high as 22% [34]. So during surgical treatment of these lesions
meticulous technique should be performed. Sinus or fistula tracts of
BCAs are typically lined by stratified squamous epithelia and some
areas may also be replaced by respiratory epithelium [35,36]. Another
lesion in the differential diagnosis of CNLs is DC which is a germ cell
tumor that results from the inclusion of embryonic epithelial
elements, and contains ectodermal and endodermal components
[21,37]. Nomenclature of these lesions is quite confusing and not
uniform. Although these lesions can be divided into epidermoid,
dermoid and teratoid cysts based on the histological findings, the
term DC has been used for all three lesions [38]. Cervical lesions
typically present 20% of head and neck dermoids and are usually
diagnosed before the age of 3 years. After careful history and physical
examination with an US, simple excision is all that is needed for cure.
However if there is an attachment of the lesion with hyoid bone a
Sistrunk’s procedure should be performed.
Figure 1
Figure 2
Figure 2
Bilateral second cleft fistula with mucoid discharge from the fistulae
openings (Arrows: fistulae openings).
Figure 3
Conclusion
TGDR, BCA and DC are common congenital neck masses in childhood. Surgical resection is optimal choice of therapy not only for aesthetic reasons but for the recurrent infections and the potential danger of malignancy. Management of CNLs may be associated with high morbidity, especially recurrence. Since CNLs are known to be complicated by infection, early referral of these patients for pediatric surgeons and accurate and timely surgical treatment is suggested.
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