The Journal of Craniofacial Surgery & Volume 23, Number 5, September 2012
24. Shaw GM, Lammer EJ, Wasserman CR, et al. Risks of orofacial clefts in
children born to women using multivitamins containing folic acid
periconceptionally. Lancet 1995;346:393Y396
Marsupialization as a Treatment
Option for the Odontogenic
Keratocyst
Reza Tabrizi, DMD,* Birkan Taha Özkan, DDS, PhD,Þ
Ali Dehgani, DMD,þ Nicole Janine Langner, DDS§
Background and Purpose: The odontogenic keratocyst (OKC) is
an unusual cyst with a high recurrence rate. The most common site
for OKCs is by far the mandible. The best treatment of OKC re-
mains controversial. Recurrence rates ranging anywhere from less
than 10% to more than 60% have been reported. The aim of our
study was to evaluate marsupialization as a treatment option for OKC.
Methods: We managed 13 patients (8 male, 5 female) between the
ages of 16 and 31 years (mean, 22.4 y) with biopsy-proven OKC.
Radiographically, the patients’ cyst sizes were between 25 and 90 mm.
Treatment consisted of marsupialization. We followed up with pa-
tients for a total duration of at least 60 months, and posttreatment
visits were carried out at 6-month intervals.
Results: We documented cured or reduced cyst size with radiogra-
phy. The odontogenic keratocyst resolved completely in 10 patients,
and the cyst walls shrank in 3 patients. The latter patients required
a second operation to remove the associated impacted teeth. A his-
tologic evaluation of 3 lesions showed metaplasia. Any case of re-
curring cysts was not seen during the entire follow-up period.
Conclusions: Marsupialization is an effective and conservative treat-
ment option for OKC. Nevertheless, future studies should conduct
even longer follow-up periods to evaluate any recurrence of lesions.
Key Words: Cyst, odontogenic keratocyst, marsupialization,
mandible
T he odontogenic keratocyst (OKC) is an unusual cyst with a high
recurrence rate. Some authors have proposed that OKC is a tu-
moral lesion because of its destructive behavior.1,2 The odontogenic
keratocyst has occurred in approximately 11% of odontogenic cysts.3
The peak occurrence of OKCs is in the second and third decades
of the patients’ lives. The peak seems to be roughly a decade youn-
From the *Department of Oral and Maxillofacial Surgery, Craniomaxillofacial
Surgery Research Center, Shiraz University of Medical Sciences, Shiraz,
Iran; †Department of Oral and Maxillofacial Surgery, Faculty of Dentistry,
University of Yuzuncuyil, Van, Turkiye; ‡Department of Oral and Maxil-
lofacial Surgery, Shiraz University of Medical Sciences, Shiraz, Iran; and
§Department of Maxillofacial Surgery, University of Innsbruck, Innsbruck,
Austria.
Received December 21, 2011.
Accepted for publication April 18, 2012.
Address correspondence and reprint requests to Dr Birkan Taha Özkan,
A?Nz Dis ve Cene Cerrahisi Anabilim DalN, Dishekimligi Fakultesi,
YuzuncuyNl Üniversitesi, Kampus/Van 65100, Turkiye;
E-mail: btozkan@hotmail.com
The authors report no conflicts of interest.
Copyright * 2012 by Mutaz B. Habal, MD
ISSN: 1049-2275
DOI: 10.1097/SCS.0b013e31825b3308
* 2012 Mutaz B. Habal, MD
Copyright © 2012 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Brief Clinical Studies
ger for women.4 Nevertheless, data have shown that OKCs are
found much more frequently in men than in women. This sex differ-
ence appears to be even more pronounced in the black community.5
One study reported that the male-to-female ratio in the black com-
munity could be as high as 5:1.6 The most common site for OKCs
is by far the mandible. Findings that range from 69% to 83% have
been reported.7 Odontogenic keratocysts that occur in regions other
than the mandibular angle seem to be more closely related to sys-
temic syndromes, especially those OKCs that occur in the maxilla.8
The best treatment of OKC remains controversial. Recurrence rates
ranging anywhere from less than 10% to more than 60% have been
reported.8 The aim of our study was to evaluate of marsupialization
as a treatment option for OKC.
CLINICAL REPORT
We managed 13 patients (8 male, 5 female) between the ages of 16
and 31 years (mean, 22.4 y) with biopsy-proven OKC. None of the
13 patients had basal cell nevus syndrome or any other medical
problems. Patients were treated in 2 centers: Taleghani Hospital,
Khorasan Razavi, Iran, and Chamran Hospital, Shiraz, Iran. All pa-
tients underwent preoperative panoramic radiographic and com-
puted tomographic scans. Radiographically, the patients’ cyst sizes
were between 25 and 90 mm. All featured OKCs occurred in the
mandible (both the anterior and the posterior). Treatment consisted
of marsupialization by excision of the overlying mucosa and open-
ing the window (91 cm in diameter) into the cystic cavity and,
whenever possible, suturing the cyst lining to the oral mucosa. In all
patients, the histologic examination was repeated a second time to
confirm diagnosis. An acrylic obturator was constructed to tempo-
rarily seal the window. Patients carried out daily irrigation of the
cystic cavity with saline solution and 0.2% chlorhexidine to prevent
a secondary infection within the cystic cavity. Patients were also
instructed to follow a strict oral hygiene. Treatment period lasted
between 7 and 16 months. Radiographies documented whether the
cysts had resolved or reduced in size. Before treatment, we informed
patients that this treatment option may need additional surgical in-
tervention. We followed up with patients for a total duration of at
least 60 months, and posttreatment visits were carried out at 6-month
intervals.
In 10 patients (76.9%), the lesions resolved completely within
the treatment period. Shrinkage of OKCs was observed in 3 patients
(23.1%), and these 3 patients required secondary operations that
allowed the surgeon to remove the associated impacted teeth. All
3 patients had multilocular lesions that occurred in the posterior of
the mandible (Figs. 1Y3). The secondary operations in all 3 patients
showed significantly increased cyst wall thickness and the eruption
of the impacted teeth associated with the cyst lesions. Initial biopsy
specimens of the 3 patients showed stratified squamous epithelium
with corrugated parakeratin at the surface and palisade basal cells
without inflammation (Fig. 4). The histologic evaluations of the le-
sions after definitive operation showed hyperplastic spongiotic non-
keratinized epithelium with granulated tissue formation of cyst walls
(Fig. 5). In 2 of 13 patients, infection occurred during treatment
period and was treated with antibiotic therapy and local care. We
FIGURE 1. Orthopantomogram view showing a huge OKC in the posterior
of the mandible.
e459
Brief Clinical Studies The Journal of Craniofacial Surgery
FIGURE 2. Orthopantomogram view demonstrating reduced size of OKC
after marsupialization but not complete resolution.
follow up with the patients at least 60 months at 6-month intervals.
Any case of recurring cysts was not seen during the entire follow-up
period. Patients had not experienced any discomfort during the
treatment period.
DISCUSSION
Odontogenic keratocysts were treated with conservative or aggres-
sive approaches. The conservative treatment generally consisted of
simple enucleation, with or without curettage, using spoon curettes
or marsupialization. Enucleation is associated with high recurrence
rates (range, 17%Y56%), especially when the cyst is removed in
fragments.9 Aggressive treatment generally includes peripheral
ostectomy, chemical curettage with Carnoy solution, and resection.5
The present study conducted decompression and marsupialization as
proposed by Brondum and Jensen.10 This treatment can also be
followed by the removal of the cyst wall after the wall has changed
through metaplasia.11,12 Previous studies have not recommended
this removal as an ideal treatment of the keratocyst because it is
believed that pathologic tissue would be left in situ.13 On the con-
trary, some studies have suggested marsupialization to allow partial
resolution and to reduce the size of the keratocyst so that the teeth or
the inferior alveolar nerve may be spared.11,14
Some authors used marsupialization alone or concomitant with
other treatment options.12,15 Recently, another study has used mar-
supialization as a definitive treatment of OKC.16 Many previous
studies have shown the successful treatment of large OKCs using
decompression and irrigation.10,17Y19 The advantages of marsupia-
lization are as follows: (1) it is more conservative than resection and
enucleation and (2) it preserves vital structures such as the inferior
alveolar nerve. In the past, a more aggressive approach was sug-
gested because of the high recurrence rate of OKCs, bone resorp-
tion, microcysts, and different modes of cyst growth.3,18
Histologic changes occurred after marsupialization; a nonkera-
tinized squamous epithelium was observed in many cases, and a
decrease in interleukin 1 and Ki-67 was also discovered in many
cases, which could be the biologic cause of the decrease in volume of
the cysts that underwent marsupialization cysts and the reduced
epithelial cell proliferation.9 Immunohistochemical studies found
higher levels of interleukin 1> (an inflammatory, multifunctional
cytokine) in OKCs than they found in dentigerous cysts. These
studies also showed that the interleukin 1> levels decrease signifi-
cantly after marsupialization.13,20 Interleukin 1> is thought to play
an important role in the expansion of OKCs by inducing the se-
cretion of keratocyst growth factor from interactive fibroblasts.13
Marsupialization also reduce interleukin 1> and cytokeratin-10,
FIGURE 3. Radiographic view after impacted teeth removal.
e460
Copyright © 2012 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
& Volume 23, Number 5, September 2012
FIGURE 4. Histologic view of the cyst before marsupialization showing
stratified squamous epithelium with corrugated parakeratin at surface and
palisade basal cells without inflammation (original magnification 400).
which were are found to be related to the expansion of the cyst.
Furthermore, the metaplasia of the epithelial lining converts into a less
aggressive form after decompression and marsupialization. However,
both techniques necessitate longer treatment periods, multiple-staged
procedures, and strict patient cooperation. The aggressive behavior
and high recurrence rate of OKC suggests a true neoplastic potential
and has therefore encouraged the World Health Organization
Working Group to classify OKC as a benign tumor with odontogenic
epithelium and mature fibrous stoma without odontogenic ectome-
senchyme.21 Although the term tumor is not synonymous with neo-
plasm because tumors also include hamartomatous tissue proliferations,
some authors believe that genetic alterations support the neoplastic
nature of OKCs. On the contrary, some clinical studies challenged this
concept by demonstrating that OKC responded well to marsupiali-
zation.22 Although this fact points to a non-neoplastic nature, some
odontogenic neoplasms may show a positive response to conserva-
tive approaches. Decompression does need a cooperative patient
who will irrigate the cyst on a regular basis and who will follow up
regularly. For this reason, only a select group of patients may be
suitable for the current treatment. A longer follow-up period is re-
quired for these patients so that practitioners can determine any
associated recurrences during this treatment.13 The worst possible
event in the long follow-up period is the recurrence of the lesion.
However, most complications of mandibular resection and imme-
diate or delayed reconstruction with bone graft and implants indicate
that the conservative treatment is a wise choice. In addition, man-
dibular resection acts as a psychologic deterrent on patients. Blanas
et al23 systematically reviewed the treatment and prognosis of OKC.
They reviewed 2290 citations pertaining to OKC, and they found only
14 investigations that met the 4 inclusion criteria. Blanas et al23 found
that resection had the lowest recurrence rate (0%) but had the highest
morbidity rate. Simple enucleation was reported to have a recurrence
rate of 17% to 56%. Simple enucleation combined with adjunctive
therapy, such as the application of Carnoy solution or decompression
before enucleation, was reported to have recurrence rates of 1% to
8.7%. However, in our study, no recurrence lesions were seen during
the follow-up period. In summary, our study showed that marsu-
pialization is an effective and conservative treatment option for
OKCs. Nevertheless, future studies should conduct even longer
follow-up periods to evaluate any recurrence of lesions.
FIGURE 5. Histologic view of the cyst after marsupialization marking
hyperplastic spongiotic nonkeratinized epithelium with granulation tissue
formation of cyst walls (original magnification 200).
* 2012 Mutaz B. Habal, MD
The Journal of Craniofacial Surgery & Volume 23, Number 5, September 2012
REFERENCES
1. Thompson L. World Health Organization classification of tumours:
pathology and genetics of head and neck tumours. Ear Nose Throat J
2006;85:74
2. Hyun HK, Hong SD, Kim JW. Recurrent keratocystic odontogenic
tumor in the mandible: a case report and literature review. Oral Surg
Oral Med Oral Pathol Oral Radiol Endod 2009;108:e7Ye10
3. Shear M. Odontogenic keratocysts: clinical features. Oral Maxillofac
Surg Clin North Am 2003;15:335Y345
4. Oda D, Rivera V, Ghanee N, et al. Odontogenic keratocyst: the
northwestern USA experience. J Contemp Dent Prac 2000;15:60Y74
5. Morgan TA, Burton CC, Qian FA. Retrospective review of treatment of
the odontogenic keratocyst. J Oral Maxillofac Surg 2005;63:635Y639
6. Shear M. Odontogenic keratocyst (primordial cyst). In: Cysts of the Oral
Regions. 3rd ed. Oxford, England: ButterworthYHeinemann,
1992:115Y119
7. Avelar RL, Antunes AA, Carvalho RW, et al. Odontogenic cysts: a
clinicopathological study of 507 cases. J Oral Sci 2009;51:581Y586
8. Woolgar JA, Rippin JW, Browne RM. A comparative histologic study
of odontogenic keratocysts in basal cell nevus syndrome and
control patients. J Oral Pathol 1987;16:124Y128
9. Giuliani M, Grossi GB, Lajolo C, et al. Conservative management
of a large odontogenic keratocyst: report of a case and review
of the literature. J Oral Maxillofac Surg 2006;64:308Y316
10. Brondum N, Jensen VJ. Recurrence of keratocysts and decompression
treatment. A long-term follow-up of forty-four cases.
Oral Surg Oral Med Oral Pathol 1991;72:265Y269
11. Marker P, BrLndum N, Clausen PP, et al. Treatment of large odontogenic
keratocyst by decompression and later cystectomy: a long-term
follow-up and a histologic study of 23 cases. Oral Surg Oral Med Oral
Pathol Oral Radiol Endod 1996;82:122Y131
12. Pogrel MA. Decompression and marsupialization as a treatment
for the odontogenic keratocyst. Oral Maxillofac Surg Clin North Am
2003;15:415Y427
13. Partridge M, Towers JF. The primordial cyst (odontogenic keratocyst):
its tumour-like characteristics and behaviour. Br J Oral
Maxillofac Surg 1987;25:271Y279
14. Rud J, Pindborg JJ. Odontogenic keratocysts: a follow-up study
of 21 cases. J Oral Surg 1969;27:323Y330
15. Tolstunov L, Treasure T. Surgical treatment algorithm for odontogenic
keratocyst: combined treatment of odontogenic keratocyst and
mandibular defect with marsupialization, enucleation, iliac crest
bone graft, and dental implants. J Oral Maxillofac Surg
2008;66:1025Y1036
16. Pogrel MA, Jordan RC. Marsupialization as a definitive treatment
for the odontogenic keratocyst. J Oral Maxillofac Surg
2004;62:651Y655
17. August M, Faquin WC, Troulis MJ, et al. Dedifferentiation of
odontogenic keratocyst epithelium after cyst decompression.
J Oral Maxillofac Surg 2003;61:678Y683
18. el-Hajj G, Anneroth G. Odontogenic keratocystVa retrospective
clinical and histologic study. Int J Oral Maxillofac Surg
1996;25:124Y129
19. Maurette PE, Jorge J, Moraes M. Conservative treatment protocol
of odontogenic keratocyst: a preliminary study. J Oral Maxillofac Surg
2006;64:379Y383
20. Ninomiya T, Kubota Y, Koji T, et al. Marsupialization inhibits
interleukin-1 alpha expression and epithelial cell proliferation
in odontogenic keratocysts. J Oral Pathol Med 2002;31:526Y533
21. Barnes L, Eveson JW, Reichart P, et al. World Health Organization
Classification of Tumours. Pathology and Genetics of Head and Neck
Tumours. Lyon, France: IARC Press, 2005:82Y89
22. Gomes CC, Gomez RC. Odontogenic keratocyst: a benign cystic
neoplasm? Oral Oncol 2007;43:619Y620
23. Blanas N, Freund B, Schwartz M, et al. Systematic review
of the treatment and prognosis of the odontogenic keratocyst.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod
2000;90:553Y558
* 2012 Mutaz B. Habal, MD
Copyright © 2012 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Brief Clinical Studies
Severe Symmetrical Facial
Lipoatrophy in a Patient With
Discoid Lupus Erythematosus
Aysin Karasoy Yesilada, MD, Kamuran Zeynep Sevim, MD,
Selami Serhat Sirvan, MD, Fatih Irmak, MD,
Hamit Soner Tatlidede, MD
Abstract: Acquired partial lipodystrophy is a rare disorder, and it
is characterized by the absence of subcutaneous fat from the face,
the neck, the trunk, and the upper extremities. The etiology of ac-
quired partial lipodystrophy includes scleroderma and discoid lu-
pus erythematosus. Literature review reveals studies involving 10
patients until today with lipoatrophy due to or after the onset of
discoid lupus erythematosus; all are female patients. We want to
report a young male patient with progressive symmetrical facial li-
poatrophy. In addition, he has discoid lupus erythematosus and ce-
liac disease. Fat grafting and adjuvant oral coenzyme Q10 tablets
(Deka-none; Deka Pharmaceuticals, Istanbul, Turkey) were admin-
istered for treatment. To our knowledge, this case involves the first
male patient in the literature presenting with symmetrical facial li-
poatrophy with very prominent periorbital lipoatrophy and bitem-
poral hollowing symptoms.
Key Words: Periorbital lipoatrophy, discoid lupus erythematosus
A youthful face is characterized by a smooth transition between
the subcutaneous compartments.1 Facial lipoatrophy presents
as a regular sign of aging in healthy individuals. However, it may
also present a sign or symptom of underlying metabolic or genetic
disorders (eg, ParryYRomberg syndrome, BarraquerYSimons syn-
drome) after cranioplasty operations or in HIV disease.2,3 Acquired
partial lipodystrophy is a rare disorder, and it is characterized by the
absence of subcutaneous fat from the face, the neck, the trunk, and
the upper extremities. The etiology of acquired partial lipodystrophy
includes scleroderma and discoid lupus erythematosus.4,5 Literature
review reveals studies involving 10 patients until today with lipo-
atrophy due to or after the onset of discoid lupus erythematosus; all
are female patients.6,7
We report a young male patient with progressive symmetrical
facial lipoatrophy. To our knowledge, this case involves the first
male patient in the literature presenting with symmetrical facial
lipoatrophy with very prominent periorbital lipoatrophy and bitem-
poral hollowing symptoms.
CLINICAL REPORT
A 26-year-old male patient presented to our clinic with lipoatro-
phy symptoms developing for the past 2 years especially on his face
From the Department of Plastic and Reconstructive Surgery, Sisli Etfal Re-
search and Training Hospital, Istanbul, Turkey.
Received January 29, 2012.
Accepted for publication April 18, 2011.
Address correspondence and reprint requests to Kamuran Zeynep Sevim,
MD, Department of Plastic Surgery, Sisli Etfal Research and Training
Hospital, Sisli, Istanbul, Turkey; E-mail: kzeynep.sevim@gmail.com
The authors report no conflicts of interest.
Copyright * 2012 by Mutaz B. Habal, MD
ISSN: 1049-2275
DOI: 10.1097/SCS.0b013e31825b33c2
e461