a case report - Otolaryngologia Polska

a case report - Otolaryngologia Polska

KAZUISTYKA / CASE REPORTS
47
Sphenoid sinus mucocele penetrating to the orbit, anterior
and middle cranial fossae and parapharyngeal space: a case report
Mukocele zatoki klinowej penetrujące do oczodołu, przedniego i środkowego dołu czaszki
oraz przestrzeni przygardłowej: opis przypadku
Rafał Chmielewski1, 2, Arkadiusz Paprocki1, Krzysztof Morawski1, Kazimierz Niemczyk1
SUMMARY
Mucocele, though is a common lesion, rarely penetrates to the surrounding
intra- and extracranial spaces. We describe the case of 45-year-old male with
2 years history of a chronic left nasal obstruction and a concentric visual field
deficit in the left eye as the only manifestations. Diagnostic CT and MRI imaging revealed a mucocele originating from the posterior ethmoid and sphenoid
sinuses and penetrating intracranially to the anterior and middle cranial fossae
and extracranially to the pterygopalatine fossa and the parapharyngeal space.
This extensive localization appears to be an extremely rare entity, which,
to our knowledge, has not been described in the English literature yet. The
clinical features and a literature review are also presented.
Hasła indeksowe: mukocele, rozrost wewnątrzczaszkowy, rozrost zewnątrzczaszkowy, dół skrzydłowo-podniebienny, przestrzeń przygardłowa, powikłania
Key words: mucocele, intracranial extension, extracranial extension, pterygopalatine fossa, parapharyngeal space, complications
©by Polskie Towarzystwo Otorynolaryngologów
– Chirurgów Głowy i Szyi
Otrzymano/Received:
30.12.2009
Zaakceptowano do druku/Accepted:
20.01.2010
1
Katedra i Klinika Otolaryngologii Warszawskiego
Uniwersytetu Medycznego, SPCSK, ul. Banacha 1a
Kierownik Kliniki: prof. dr hab. med.
K. Niemczyk
2
Zakład Anatomii Prawidłowej i Klinicznej
Centrum Biostruktury Warszawskiego
Uniwersytetu Medycznego, ul. Banacha 1 a,
02-097 Warszawa
Wkład pracy autorów/Authors contribution:
Rafał Chmielewski zebrał materiał, opracował dane
i napisał pracę, Arkadiusz Paprocki był lekarzem
prowadzącym chorego i konsultował zebrany
materiał, Krzysztof Morawski i Kazimierz Niemczyk
byli operatorami oraz konsultowali pracę na
wszystkich jej etapach powstawania.
Konflikt interesu/Conflicts of interest:
Autorzy pracy nie zgłaszają konfliktu interesów.
Adres do korespondencji/
Address for correspondence:
imię i nazwisko: Rafał Chmielewski
adres pocztowy:
Katedra i Klinika Otolaryngologii WUM
ul. Banacha 1 a
02-097 Warszawa
tel. 0-22 599 25 21
fax 0-22 599 25 23
e-mail [email protected]
Introduction
Case report
Mucocele is a benign condition, still of controversial
etiology. The variety of factors may lead to occlusion
of the paranasal sinus mucous membrane gland
ostium, causing the secretion accumulation. It results in development of the lesion with a potential
for adjacent bony remodeling and resorption. Untreated cases have a tendency for spreading to the
surrounding intra- and extracranial spaces, causing
orbital complications (visual loss, gaze restrictions
and proptosis), cerebrospinal fluid (CSF) leak and,
in the advanced stage, the mass effect on the brain
with accompanying neurological symptoms. We report
on the case of mucocele of the posterior ethmoid and
sphenoid sinuses penetrating intracranially to the
anterior and middle cranial fossae and extracranially
to the pterygopalatine fossa and the parapharyngeal
space. This extensive localization appears to be an
extremely rare entity, which, to our knowledge, has
not been described in the English literature yet.
The clinical features and a literature review are
also presented.
A 45-year-old male patient presented to our ENT
Department with 2 years history of chronic left nasal
obstruction and concentric visual field deficit in the
left eye as the only manifestations. Before the presentation he was initially diagnosed with a chronic
rhinosinusitis and a glaucoma of the left eye, both of
which being treated conservatively. On the physical
examination he demonstrated significantly compromised left nasal cavity, bulging of the lateral nasopharyngeal wall on the left side and a concentric visual
field deficit in his left eye. Computed Tomography
(CT) revealed a tumor of the left middle cranial fossa
with destruction of the greater wing of the sphenoid
bone and the apex of the petrous part of the temporal
bone, lateral clivus, lateral wall of the sphenoid sinus
and the walls of the orbital apex. It was filling the
left sphenoid and posterior ethmoid sinuses. In its
extension to the skull base it was distending the left
pterygopalatine fossa and transposing anteriorly the
posterior wall of the left maxillary sinus. Inferiorly it
reached the left parapharyngeal space and bulged out
O t o l a r y ngo l og i a Po l sk a t o m 6 4 , nr 1, s t yc ze ń – l ut y 2 010
Otolaryngol Pol 2010;
64 (1): 47-49
48
KAZU ISTYKA / CASE REPORTS
Fig. 1. MRI of the mucocele in the axial plane. Well defi ned lesion in the size of 67x41x66 mm in the left posterior
ethmoid, sphenoid and most of the left middle cranial
fossa. In both T1 and T2 weighted images the lesion is hyperintensive and is not enhanced by the contrast medium.
The signal intensity of the adjacent brain tissue is not
changed.
Ryc. 1. Mukocele w obrazie rezonansu magnetycznego,
płaszczyzna pozioma. Dobrze odgraniczona zmiana o wymiarach 67x41x66 mm w okolicy lewego tylnego sitowia oraz zatoki klinowej i lewego dołu środkowego czaszki. W projekcjach
T1 i T2 zależnych zmiana jest hiperintensywna i nie wzmacnia
się po podaniu kontrastu. Sygnał otaczającej tkanki mózgowej
pozostaje niezmieniony.
Fig. 2. MRI of the mucocele in the sagittal plane.
Ryc. 2. Mukocele w obrazie rezonansu magnetycznego,
płaszczyzna strzałkowa.
the superior and lateral walls of the nasopharynx. The
tumor density of 30 Hounsfield units and capsular
enhancement were characteristic for the mucocele.
Magnetic Resonance Imaging (MRI) confirmed the
localization and the morphology of the tumor and
determined the exact size – 67x41x66 millimeters
(Figs. 1, 2). In both T1 and T2 weighted images the
lesion was hyperintensive and did not show any enhancement. The adjacent brain tissue was normal.
Nasal septum was deviated to the right.
The endonasal microscopic ethmoidectomy with
excision of the left middle nasal concha and marsupialization of the mucocele wall was performed. The
postoperative period was uneventful with an immediate
full recovery from the left nasal and eye symptoms. The
patient was released home 2 weeks after the surgery.
He has been followed in our outpatient clinic at regular intervals for 4 years now and underwent yearly a
complete reevaluation with the clinical assessment,
sinonasal endoscopy, CT and MRI imaging (Figs. 3, 4).
On endoscopy there is a healthy mucous membrane
lined cavity widely open to the inferior and middle
nasal meatus. Until now, no signs of recurrence nor
other complications have been found and the patient
remains asymptomatic.
Discussion
Mucoceles are common sinus lesions, frequently
affecting the frontal and ethmoid sinuses, being less
common in the maxillary sinuses, but rarely located
in the sphenoid sinuses. Their frequency in the sphenoid sinus is presented by many authors at the level of
1-3% [1, 4], but some report higher incidence of 8.8%
out of 57 cases – [2], 10% out of 60 cases – [7] or
even as high as 18.75% out of 16 cases [3]. Kösling et al. [11] after Hejazi state that by 2001, there
have been 130 sphenoid sinus mucocele described in
the literature. In the literature review presented by
Yokoyama et al. [1], of the total 188 mucocele cases
reviewed, 11.2% had an intracranial extension. Most
commonly it was to the suprasellar and anterior
cranial fossa regions, but none of those cases had a
mucocele penetrated through the skull base. Sautter
et al. [2] analyzed 57 cases of mucoceles with either
the skull base or the orbital walls erosion. The orbit
was affected in 51% of cases, skull base in 40% and
in the remaining 9% both regions were eroded. The
extracranial extension of the mucocele has not been
described in this group of patients. Koike et al. [12]
mentions, that the mucocele causes the skull base
destruction in about 10% to 20% of cases. Serrano
in his report of 60 mucocele cases [13] reported 10%
of sphenoid sinus mucocele in which two thirds were
affecting only the sphenoid sinus and one third both
the sphenoid and ethmoid sinuses were involved.
O t o l a r y ngo l og i a Po l sk a t o m 6 4 , nr 1, s t yc ze ń – l ut y 2 010
KAZUISTYKA / CASE REPORTS
Fig. 3. CT of the head after the mucocele marsupialization
(axial plane). Bony dehiscence in the lateral wall of the left
sphenoid sinus, greater wing, orbital apex and the apex of
the petrous part of the temporal bone corresponds to the
lesion extent in the preoperative CT scan. Base of the left
middle cranial fossa is filled with the cerebrospinal fluid. No
signs of the brain tissue nor meningeal pathological enhancement. Brain ventricles are of the proper shape and position.
Ryc. 3. Obraz tomografii komputerowej głowy po zabiegu
(płaszczyzna pozioma). Ubytek kostny w bocznej ścianie lewej
zatoki klinowej, skrzydle większym kości klinowej, szczycie
oczodołu oraz szczycie piramidy kości skroniowej odpowiada
zasięgowi mukocele w przedoperacyjnym obrazie tomografii
komputerowej. Podstawa lewego dołu środkowego czaszki
wypełniona płynem mózgowo-rdzeniowym. Bez cech patologicznego wzmocnienia opon mózgowo-rdzeniowych i tkanki
mózgowej. Komory mózgu o prawidłowym kształcie i położeniu.
The authors points out the importance of the history
of the sinus diseases and sinus surgeries as a pathological factor of the mucocele development. 81.7
% of the cases they analyzed had a sinus disease
history, 45% had sinus surgeries performed before
their presentation. All of the sphenoid sinus mucocele
cases were treated by the functional endoscopic sinus
surgery (FESS). From the group of previously operated patients, the mucocele developed earlier after
FESS (mean 2 years) than after the open approaches
(mean 18 years). In MEDLINE search (1965-2009)
we found 2 papers mentioning the existence of the
nasopharyngeal and parapharyngeal extensions of
the mucoceles, without giving the descriptions of cases [7, 9]. Cerqua et al. [9] mentioned three sphenoid
sinus mucocele cases, two of which with destruction
of the sellar f loor and upward invasion, one with
posterior invasion and involvement of the clivus. In
his experience, sphenoidal mucoceles tend to spread
more frequently in an anterior-inferior fashion with
invasion of the ethmoid sinus, nasal cavity and the
nasopharynx, but rarely penetrate to the middle or
O t o l a r y ngo l og i a Po l sk a t o m 6 4 , nr 1, s t yc ze ń – l ut y 2 010
Fig. 4. MRI of the head after the mucocele marsupialization
(coronal plane). Base of the left middle cranial fossa is filled
with the cerebrospinal fluid. No signs of the meninges, brain
tissue, nor sphenoid sinus mucous membrane pathological
enhancement. Brain ventricles are of the proper shape and
position.
Ryc. 4. Obraz rezonansu magnetycznego głowy po zabiegu
(płaszczyzna czołowa). Podstawa lewego dołu środkowego
czaszki wypełniona płynem mózgowo-rdzeniowym. Bez cech
patologicznego wzmocnienia opon mózgowo-rdzeniowych
i tkanki mózgowej, czy błony śluzowej zatoki klinowej. Komory
mózgu o prawidłowym kształcie i położeniu.
posterior cranial fossae. To our knowledge, the case
we present in this paper, has not been described in
the English literature yet.
In the differential diagnosis of the extracranial
extension of the mucocele we should be aware of such
cases like the cyst of the nasopharynx commonly called the “Tornwaldt’s cyst” [6]. This lesion is originating
from the nasopharyngeal roof from the embryonic
remnants of the Rathke’s pouch. The CT and MRI
findings in the nasopharyngeal cyst can be similar,
but contrary to mucocele, no association with the
paranasal sinus lesion are present. The intracranial
mucocele extensions should be differentiated with the
meningeal and the brain tissue tumors.
Over the last two decades it has been commonly
accepted that the intranasal endoscopic/microscopic
approach with the mucocele wall marsupialization
is the method of choice for the treatment in these
lesions. Before that time, the open approaches were
used, which were associated with significantly higher
risk of severe intra- and postoperative complications
[8, 10].
In conclusion, the mucocele is not a rare entity, but
because of its localization and extension can present
an intriguing diagnostic and therapeutic puzzle to
solve.
49
50
KAZU ISTYKA / CASE REPORTS
REFERENCES
1.
6.
Yokoyama T, Inoue S, Imamura J, Nagamitsu T, Jimi Y,
Katoh S, Kameda H, Abiko S Sphenoethmoid Mucoceles
with Intracranial Extension – Three Case Reports. Neurol
326.
7.
Med Chir (Tokyo) 1996; 36, 822-828, 1996.
2.
12(6): 527-530.
8.
endoscopic approach sufficient? Otolaryngol Head Neck
Kawaguchi S et all Giant frontal mucocele extending into
the anterior cranial fossa. J Clin Neurosci 2002 9(1), 8689.
4.
Wang L, Kim J, Heilman CB Intracranial Mucocele as a
Complication of Endoscopic Repair of Cerebrospinal Fluid Rhinorrhea: Case Report. Neurosurgery 1999; 45(5),
1243-1245.
5.
Lim SA, Sitoh YY, Lim TC, Lee JC Clinics in diagnostic
Har-El G, Balwally AN, Lucente FE Sinus mucoceles: Is
marsupialization enough? Otolaryngol Head Neck Surg
Surg 2008; 139, 570-574.
3.
Iqbal J, Kanaan I, Ahmed M, Homsi M Neurosurgical aspects of sphenoid sinus mucocele. Br J Neurosurg 1998;
Sautter NB, Citardi MJ, Perry J, Batra PS Paranasal sinus
mucoceles with skull-base and/or orbital erosion: Is the
Gospodarek T, Betlejewski S A case of mucous cyst of the
nasopharynx. Otolaryngol Pol 1997; 51 Suppl 25: 324-
1997; 117: 633-640.
9.
Cerqua N, D’Ottavi LR, Coen-Tirelli G, Piccirillo E Mucocele of sphenoid sinus: presentation of 3 cases. Acta Otorhinolaryngol Ital. 1996; 16(1): 52-56.
10. Haloi A. K, Ditchfield M, Maixner W Mucocele of the sphenoid sinus. Pediatr Radiol 2006; 36: 987-990.
11. Kösling S, Hintner M, Brandt S, Schulz Th, Bloching M
Mucoceles of the sphenoid sinus. Eur J Radiol 2004; 51:
1-5.
imaging (120). Right rhinogenic optic neuritis secondary to
12. Koike Y, Tokoro K, Chiba Y, Suzuki S, Murai M, Ito H
mucocele of the Onodi cell. Singapore Med J. 2008; 49(1),
Intracranial extension of paranasal sinus mucocele: two
84-87.
case reports. Surg Neurol 1996; 45: 44-48.
O t o l a r y ngo l og i a Po l sk a t o m 6 4 , nr 1, s t yc ze ń – l ut y 2 010