nasal deformities are rare, occurring in 1 in 20,000 to 40,000 newborns (1).
These lesions include nasal cysts, fistulas, tumors and pits that have their
origin in the ectodermal or neuroectodermal layers. An incorrect embryologic
development of the nose in a phase of the nasal and anterior rhinobase
separation constitutes the nasal anomaly.
neuroectodermal anomalies include gliomas, meningoceles
and meningoencefaloceles, and the ectodermal forms represent dermoid cysts
with or without fistulas and dermoid nasal sinus cysts. The nasal dermoid
cyst is a cystic formation occurring in the middle of the face and is commonly
related to the nasal dorsum, glabella or medial canthus, usually without
intracranial propagation. The dermoid nasal sinus cyst is a cystic mass
spreading under the nasal dorsum to the foramen caecum with possible
intracranial and extradural extension (2). Both should be differentiated from
encephaleceles in this region.
are two theories to interpret the pathogenesis of congenital ectodermal defects
in the region of the nose and the anterior rhinobase. The cranial theory points
to a prenasal space and differentiation of the rhinobase, where the remaining
contact of the nasal skin and dura create a cystic tract with a dural
attachment. The superficial theory, in contrast, identifies a failure of the
ectodermal extension, with the ectodermal sticking between the two medial nasal
processes, which leads to the creation of a sinus or a cyst (3).
dermoids manifest themselves with nasal swelling and usually contain skin
adnexa (e.g. hair follicles or sebaceous glands). The clinical examination of
patients with nasal fistula reveals a punctiform skin defect on the nasal
dorsum with intermittent detritus discharge. In addition, nasal dermoid cysts
often present with inflammation.
deformation of the external nose such as nasal swelling, elevated nasal dorsal
projection, nasal gibbus or nasal fistula are found after birth, or spotted a
little later by parents or paediatricians (4, 5).
imaging is necessary to arrive at the proper diagnosis. A CT scan defines the
osseal borders or the bony defect, and the size of the enlargement. MRI is
needed to exclude the potential for intracranial extension, focusing on the
meningeal integrity and the contact with frontal lobes. The only therapeutic
option is surgery – complete excision is necessary to avoid a recurrence. There
are various opinions in the literature about the best surgical approach and the
timing of the operation. Treatment of the common dermoid cyst is usually not
difficult but can be sporadic in adolescents and adults. Nasal dermoids can be
removed through transcolumellar incision, as in open structure rhinoplasty, and
osteotomy may also be required. The transfacial and transcranial approach,
combined with the endoscopic transnasal approach, can be used as well.
Neurosurgical evaluation is needed in case of suspected intracranial extension.
patients with dermoid nasal cysts and cutaneous fistula on the nasal dorsum are
MATERIALS AND RESULTS
We evaluated the
diagnostic process and the surgical therapy effect at the University ENT
Department, Bratislava, Slovakia during the years 2006–2009. We identified
three patients, aged 15, 17 and 18, with nasal dermoid (1 girl and 2 boys) for this retrospective
review. All of them had prior surgeries at different hospitals. None of the
subjects had other associated anomalies, or intracranial extension.
15-year-old girl underwent two preceding excisions of a nasal fistula. She
presented with recurrent nasal swelling underneath the scar (Fig. 1). CT scan
revealed a cystic formation between the frontal sinuses. The cyst has been
clearly separated from the intracranium, and its canal continued to the nasal
dorsum and the scar on the skin (Fig. 2.)
total resection of both the fistula with the canal and the cyst was performed
via dorsal incision. Part of the anterior wall of the frontal sinuses was
opened, and after the medial osteotomy was preformed, the cavity was filled in
with fat tissue (Fig. 3).
A recurrent nasal
enlargement after the resection of a nasal fistula (performed at
a different hospital) was found in an 18-year-old boy (Fig. 4). The CT
scans exposed the cystic formation under all the nasal bones without
intracranial extension (Fig. 5). The cyst was removed using open rhinoplasty;
the opening of the fistula on the skin stayed intact (Fig. 6).
A couple of exstirpations
of the nasal fistula were performed in a 17-year-old boy during his childhood
(Fig. 7A). A residual opening with intermittent discharge formed on the nasal
dorsum (Fig. 8). The CT scan showed a cystic formation without
intracranial extension under the nasal bone. The open rhinoplasty technique
with a resection of the dermoid cyst and its canal was performed with a gibbus
resection and four osteotomies (Fig. 9). The skin scar stayed intact (Fig. 7B).
reports about nasal dermoids and their management are abundant in the medical
literature. (6, 7, 8, 9). The optimal age for surgery is during the 5th and 6th
years of life, when the risk of leaving a residual dermoid tissue is not as
high (7). Exstirpation with an external, transfacial incision was commonly
performed in the past for patients without intracranial involvement, but
nowadays the transcolumellar incision with open rhinoplasty is recommended (10,
11, 15). This has become the standard procedure with regard to the functional
and aesthetic aspects of the nasal surgery. Transnasal endoscopic resection is
another option. If the nasal dermoid lies under the nasal bones and glabella,
endoscopic procedures might be complicated. Moreover, transnasal endoscopic
resection is generally not recommended for dermoids extending into or beyond
the falx cerebri, or other brain structures (1).
Heywood et al. (12)
suggest the use of a brow incision and a small window craniotomy as a
successful low morbidity technique for the excision of nasal dermoids with
For the surgical
removal of nasal dermoids with intracranial extension, both the transglabellar
subcranial approach (13) and the bicoronal incision with craniotomy (14) are
effective. A total excision prevents a recurrence, but the cosmetic
aspects of the excision have to be considered.
In the first case, an
external approach directly through the fistulous aperture was used because the
surrounding tissue was inflamed and scarred. The skin had to be excised and then
sutured. After healing, the scar on the nasal dorsum was not very noticeable.
We do not have post-surgical photographic documentation, as the patient did not
come in for her 6-month follow-up visit. For the other two patients
transcollumelar incision together with an open rhinoplasty was chosen. This
approach is more convenient if the external orifice of the fistula is located
inferiorly, or if the nasal dorsum has to be corrected due to being widened by
an expansive cyst growth.
The open approach
provides a sufficient overview in an operative context, and it makes it
possible to perform osteotomies in order to narrow the nasal pyramid. The cyst
and fistula can be completely removed, and the aesthetic result is good. The
excision of the cyst can create a new preformed cavity which is not
connected to the paransal sinuses and their draining system with the
respiratory epithelium. In these cases, obliteration of such a cavity
seems to be a good option. In order to perform fine exploration of nasal
dermoid with a pleasing cosmetic effect, Bloom et al. (15) recommend
external rhinoplasty as the surgical approach of choice. Different surgical
techniques consist of the direct median approach and the paracanthal approach
otorhinolaryngologists usually deal with the surgical treatment of the nasal
dermoid cysts and fistulas. These procedures are relatively rare
in adolescents and adults. Delayed diagnosis may be caused by previous
improper treatment. If a patient underwent excision of the external orifice
and curretage of a fistula without having pre-operative imaging,
physicians usually do not consider the diagnosis of a congenital nasal dermoid
cyst. Cysts diagnosed later in life have a higher frequency of deep involvement
and therefore require more extensive surgery (17).
Nasal dermoids must be
differentiated from gliomas or encephaloceles, and the diagnosis must be
confirmed with a CT scan or an MRI. An MRI is considered the most accurate way
of evaluating nasal dermoids and is also essential for preoperative planning.
Dermoid cysts are the
most common midline congenital nasal masses and may extend intracranially. To
make the proper diagnosis, radiological imaging with an evaluation of the
intracranial structures should be obtained. Total exstirpation with an
acceptable cosmetic result is the only causal therapy. Open rhinoplasty is one
of the appropriate methods in patients without dural attachment or intracranial
propagation of the nasal dermoid.
Prof. Pavel Doležal, M.D., PhD
of the E.N.T. Dept., Slovak Medical University
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