Lupine Publishers | Journal of Otolaryngology Research Impact Factor
Abstract
Congenital cysts, sinuses and fistulas of the head and neck are
anomalies that may be encountered in pediatric life. They
represent abnormal development of the brachial apparatus or inclusion of
the ectoderm at any site of the lines of fusion of the face.
In the head, preauricular, salivary and median nasal fistulas as well as
dermoid cysts and Van der Woude syndrome are examples.
In the neck, median thyroglossal cyst and fistula, and lateral brachial
cyst and fistula are well-known congenital anomalies. Proper
diagnosis of these malformations is essential to decide the line of
treatment. Standard and modified surgical procedures are adopted
for excision and repair of these anomalies with the possibility of
incidence of complications, recurrence and malignancy.
Keywords: Congenital; Cysts; Sinuses; Fistulas; Craniofacial; Cervical; Thyroglossal; Branchial
Abbreviations: CT: Computed Tomography; MRI: Magnetic Resonance Imaging; MULFs: Median Upper Lip Fistulas; TGDCs:
Thyroglossal Duct Cysts; VWS: Vander Woude Syndrome
Introduction
A fistula is an abnormal tract communicating the cavities
of two internal organs or the cavity of an organ with the body
surface whereas a sinus is one-end blind tract that opens on the
surface. Congenital cysts, sinuses and fistulas of the head and neck
are uncommonly detected in children. They include congenital
craniofacial and cervical anomalies; the latter may be mediocervical
or latero-cervical in position. Surgical excision of these
lesions is the only option of treatment.
Congenital Craniofacial Cysts and Fistulas
Congenital periauricular cysts, sinuses and fistulas occur
commonly in children are developmental anomalies of the first
branchial cleft and first pharyngeal arch [1,2]. Children with
recurrent postauricular abscesses show associated congenital
anomalies like postauricular sinuses, post auricular dermoid
cysts, first branchial cysts, aural fistulas and aural atresia [3].
Congenital salivary fistulas of the main part of the parotid gland are
rarely reported; surgical excision of the fistula and parotid gland
is an effective line of treatment for this anomaly [4]. Odontogenic
cutaneous fistula results from abnormal tooth canalization caused
by chronic periapical infection; it may be wrongly diagnosed as a
congenital fistula [5]. Abnormal protrusion of a part of the brain and
its meninges through a defect in the skull bones is termed meningoencephalocele.
A case of an anterior meningo-encephalocele
presenting as a small swelling in the right lower eyelid was
reported; if it was mistreated as a lachrymal swelling it might result
in meningitis and cerebrospinal fluid leakage [6]. The nasal dermoid
cyst and median nasal sinus are rare congenital anomalies that are
attributed to early embryonic inclusion of the ectodermal germ
layer during closure of the anterior neuropore of the neural tube
[7]. Congenital nasofrontal fistulas (also termed nasofrontal dermal
sinuses) are extremely rare malformations that may be complicated
with serious meningitis [8]. Patients with congenital nasal fistulas
and dermoid cysts are operated upon through vertical midline or
horizontal glabellar, and nasofrontal osteotomy [9]. A craniofacial
dermoid cyst at the lateral wall of the orbit was reported to be
associated with a cutaneous frontotemporal fistula that extended
to the dura of the temporal lobe of the brain through the temporal
and sphenoidal bones; lateral orbitotomy was surgically performed
in this case [10].
Congenital sinuses and fistulas of the lip are uncommon and
median upper lip fistulas (MULFs) are extremely rare. A case of
congenital MULF, at the philtrum of a girl infant, was surgically
removed. The fistulous tract was histological lined with squamous
epithelium that contained sebaceous and mucous glands as well as
hair follicles. [11,12] Van der Woude syndrome (VWS) is a single
gene autosomal dominant congenital craniofacial abnormality that
is featured by labial cysts, accessory salivary glands, and lower lip
pits, fistulas and paramedian sinuses; it is commonly associated
with cleft lip or palate or both [13,14]. Excision of lower lip sinuses
and accessory glands, reconstruction of the lip, nose, and associated
cleft lip and palate are the components of VWS surgery [15].
Surgical excision of congenital lower lip sinuses has an increased
rate of mucoceles formation and recurrence [16].
Congenital Cervical Cysts and Fistulas
Neck masses are frequently met with in children and they
may be congenital or acquired in origin. Congenital neck swellings
include branchial cysts, thyroglossal cysts, hemangiomas and
cystic hygromas. They may be complicated into sinuses and
fistulas [17,18]. Congenital cervical cysts, sinuses and fistulas are
not common. Thyroglossal duct cysts (TGDCs) are the commonest
malformations followed, in frequency, by branchial cleft anomalies
and dermoid cysts [19]. Congenital cysts and fistulas of the neck
are divided, according to their locations, into midline or mediocervical
and latero-cervical anomalies [20,21]. In man during
the 3rd to the 4th week of development, a pharyngeal (branchial)
apparatus is formed in the wall of the pharyngeal gut. It consists of
six mesodermal arches, four ectodermal clefts and five endodermal
pouches. The derivatives of these branchial elements include
essential structures in the head and neck while their remnants
may develop into congenital neck anomalies like branchial cyst
(and fistula) and thyroglossal cyst (and fistula) [22]. Laterocervical
congenital cysts and fistulas are mentioned to be due to
abnormal development of branchial clefts particularly the 2nd one;
they are usually found at the anterior border of the lower third
of the sternocleidomastoid muscle. The TGDCs (and fistulas) are
the most common medio-cervical congenital anomalies; they are
usually found in the median thyrohyoid region. Surgical resection
of the cysts and fistulas should be complete to avoid recurrence
and risk of malignancy [23]. Congenital cervical salivary fistulas
result from ectopic non-branchial salivary tissue that has a sinus
draining serous or mucous secretion to the anterior part of the base
of the neck [24]. In congenital dermoid fistula of the anterior chest
region, a skin orifice could be seen at the anterior border of the
sternocleidomastoid muscle with subcutaneous extension of the
fistulous tract till the sternoclavicular joint [25].
Median Neck Cysts and Fistulas
TGDCs are the commonest congenital swellings of the neck
[26]. They represent 2% of cervical masses and 70% of congenital
neck swellings [27,28]. TGDCs result from patency of a part of the
embryonic thyroglossal duct that extends from the foramen cecum
(on the dorsum of the tongue) till the thyroid primordium (pouches).
They are painless and commonly diagnosed in children below the
age of 5 years. A TGDC may ulcerate spontaneously leading to the
formation of a thyroglossal fistula that rarely communicates with
foramen cecum [28]. Thyroglossal fistulas may be suprahyoid,
hyoid or infrahyoid in position [29]. It is agreed that congenital
mento-sternal fistulas are embryologically and pathologically
different from thyroglossal fistulas. Mento-sternal fistulas are most
probably caused by midline cervical inclusion of ectodermal or
endodermal tissue during fusion of the branchial elements [30].
Excision of the TGDC and fistula is performed through Sistrunk
procedure which includes removal of the body of the hyoid bone.
The lining epithelium of the excised cysts varies according to the
sites of the cysts; it is cuboidal, columnar, pseudo stratified or
stratified squamous in type [31]. In an adult female patient, during
a Sistrunk procedure for a TGDC with recurrent purulent discharge,
two fistulous tracts terminating at the hyoid bone were discovered;
it was mentioned that similar cases were missing in the literature
[32]. Moreover, a branched thyroglossal duct terminating in two
separate cysts with a chronic fistula was diagnosed in a young
woman with a history of an incomplete surgical excision of a TGDC
[33].
Clinically, most of the thyroglossal fistulas are considered as
the squeal of interventional procedures: incisions, punctures,
enucleations or radiations of TGDCs. Radical Sistrunk procedure
is an effective option for removal of a TGDC [34-37]. Standard or
modified Sistrunk operation includes removal of the TGDC and/or
fistula together with the middle part of the hyoid bone; the duct
is microscopically or macroscopically dissected till the foramen
cecum to avoid recurrence [38-43]. The recurrence rate of TGDCs
removed by Sistrunk procedure is reported to be 4% [44]. In a
group of patients with TGDCs and fistulas, the size of the cyst varied
from 1 to 4 cm; Sistrunk procedure had the advantages of low rates
of complications (9.08%) and recurrence(1.82%), avoidance of
antibiotics usage and short hospital stay [45]. Preoperative neck
ultrasonography is recommended as a diagnostic tool of a TGDC
whereas postoperative histopathology of the excised cyst and
fistula is mandatory to exclude rare occurrence of malignancy
[46,47]. An unusual case of a TGDC at the base of the tongue and
fistulizing to the anterior part of the tongue was described and
successfully excised via a combined trans-hyoid and intra-oral
surgical approach [48].
Piriform sinus fistulas are congenital anomalies of the third and
fourth branchial arches. They are usually unilateral and associated
with thyroiditis and recurrent abscesses in the lower part of the
front of the neck. A case of rare bilateral congenital piriform sinus
fistulas is mentioned in the literature [49].
Lateral Neck Cysts and Fistulas
Branchial anomalies of the 1st to 4th branchial clefts and arches
represent 20% of all congenital head and neck swellings in children
[50-52]. Branchial cleft anomalies can present as a neck swelling,sinus,
or fistula. Second branchial cleft anomalies account for
about 95% of all anomalies of the branchial apparatus. Repeated
preoperative infections of these anomalies lead to a higher rate
of postoperative recurrence [53]. Branchial cysts are supposed to
result from incomplete developmental obliteration of the branchial
cleft or trapping of cell nests in the branchial apparatus that can
later
form branchial cysts [54]. Second branchial cleft cysts and sinuses
are the most common type of branchial cleft anomalies. Failure of
obliteration of the cervical sinus of His leads to the formation of a
branchial cyst and failure of fusion of the 2nd branchial arch with the
5th arch results in a branchial sinus (fistula) [55]. A rare coexistence
of TGDC and branchial cyst with fistula in the same child is reported
in the literature [56]. Unilateral first and bilateral second branchial
cleft fistulas were concomitantly observed together with a
preauricular sinus and moderate bilateral hearing loss in branchiootic
and branchio-oto-renal syndromes [57]. A case of an ectopic
tooth in a branchial cleft anomaly was described in a young girl
who had Townes-Brocks syndrome [58]. Another case of second
branchial cleft sinus remained asymptomatic till the sixth decade of
life when it was surgically excised together with ipsilateral carotid
endarterectomy [59]. Furthermore, a congenital branchial fistula
may be complete with an internal orifice at the tonsillar fossa;
complete surgical excision of the fistulous tract could be performed
in one setting [60].
Conclusion
Congenital cysts, sinuses and fistulas of the head and neck
must be properly diagnosed and managed to diminish the rate of
complications and recurrence, and to avoid the risk of malignancy.
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