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Background: Exenteration is indicated in patients with malignant
neoplasms of orbital contents. It entails the removal of the
eyeball together with its extraocular muscles and other soft
tissues. Exenterations can be classified into (1) total, (2)
subtotal, and (3) supertotal exenteration. Retrospectively
study, we reviewed 7 patients that had received exenteration/subtotal
exenteration with spontaneous granulation/myocutaneous flap
implantation or eyelid-sparing exenteration with myocutaneous
flap. Primary lesions, histopathological examination results,
treatments, and recurrences are discussed.
Methods: A retrospective study of the years 1987 through 2000
disclosed 7 patients that underwent exenteration/subtotal
exenteration. The patients ranged in age from 41 to 68 years.
Two patients underwent total exenteration without socket augmentation;
4 patients underwent exenteration/ subtotal exenteration with
immediate facial reconstruction, and 1 with delayed facial
reconstruction.
Results: Classification of the 7 patients showed that 2 had
basal cell carcinoma of the skin, 2 had squamous cell carcinoma
of the conjunctiva, 1 had squamous cell carcinoma of the paranasal
sinus, 1 had rhabdomyosarcoma of the paranasal sinus, and
1 had intracranial meningioma. Radiotherapy was performed
in 6 of the patients and chemotherapy in 2. Central nerve
system invasion was noted in 2 patients, and 1 died due to
it.
Conclusion: Secondary orbital tumors involved the orbit from
adjacent tissues: paranasal sinuses, nasopharynx, lacrimal
sac, conjunctiva, eyelid, intraocular tissue, and intracranial
tissues. Combined surgeries are necessary for complete tumor
removal. And the imaging studies should include the field
of the orbit, sinus, and brain to search for the primary lesions.
(Chang Gung Med J 2002;25:599-605)
Key words: exenteration, secondary orbital tumors, split
- thickness skin graft, basal cell carcinoma, squamous cell
carcinoma, rhabdomyosarcoma.
Secondary orbital tumors represent one third of all orbital
tumors.(1) They arise in adjacent tissues including sinuses,
nasopharynx, meninges, brain, intraocular, conjunctiva, lid,
and lacrimal sac but most secondary orbital tumors arise from
the paranasal sinus, especially the maxillary sinus. The signs
are as follows: proptosis, global displacement, conjunctival
congestion and chemosis, squinting, decreased acuity, and
nasal obstruction. We present seven patients with secondary
orbital tumors, who underwent total or eyelid-sparing exenteration.
We emphasize the survey for the adjacent orbital tissue to
make early diagnosis and combined therapy for the highly malignant
tumors.
METHODS
A retrospective search of the years from1987 through 2000
identified 7 patients who underwent orbital exenteration.
The patients ranged in age from 41 to 68 years. An ophthalmologist,
otolaryngologist, or plastic surgeon performed the surgeries.
Two patients underwent total exenteration with spontaneous
granulation; 4 patients underwent exenteration/subtotal exenteration
with myocutaneous flap or split - thickness skin graft (STSG),
and 1 underwent exenteration with delayed myocutaneous flap.
RESULTS
In our retrospective study, the 7 patients ranged in age
from 41 to 68 years. They all underwent tumor excision with
total exenteration with spontaneous granulation (2 patients),
total exenteration with delayed myocutaneous flap (1 patient),
total/subtotal exenteration with myocutaneous flap (4 patients).
Histopathological examination results revealed that 2 patients
had basal cell carcinoma (BCC) (skin), 2 had squamous cell
carcinoma (SCC) (conjunctiva), 1 had SCC (maxillary and ethmoid
sinuses), 1 had rhabdomyosarcoma (maxillary and ethmoid sinuses),
and 1 had meningioma (brain). Two patients showed central
nervous system (CNS) invasion and one died due to it. One
showed optic nerve invasion. Radiotherapy was performed on
5 patients and chemotherapy on 2 patients. Four patients showed
recurrence before exenteration and 1 recurrence happened after
exenteration. Two showed brain metastasis; one showed liver
metastasis.
Case 1
A 62-year-old man had BCC over his right forehead, which was
diagnosed at a different hospital. Twelve months later, he
was presented at our hospital due to a recurrent mass over
his forehead on the right side, size. Computed tomography
(CT) revealed a subcutaneous soft tissue mass over his forehead
on the right side. After wide excision of the tumor mass by
plastic surgeon, BCC was diagnosed according to pathologic
examination results. Three months after excision, recurrent
extracranial mass over the temporal area was noted. Further
surgery involving wide excision and temporal adipofacial flap
with STSG was performed. Five years after excision, at regular
follow-up, CT revealed orbital and outer dura invasion. Total
exenteration accompanied by myocutaneous flap was performed.
The patient underwent fractioned radiotherapy (6660 cGy/37)
and no recurrence was noted after exenteration and radiotherapy,
during 11 months follow-up.
Case 2
A 67-year-old woman with a huge mass over the left medial
canthus and nose for more than 10 years and came to visit
our clinic. CT revealed a 6.5 cm irregular fungating mass
in the left periorbital, glabellar, and nasal regions, which
impressed skin cancer. Palliative tumor excision was performed
by otolaryngologist doctor and the histopathologic examination
results were basal cell carcinoma. Three months after excision,
tumor regression was noted. But due to the possible residual
tumor, electron beam therapy was performed during follow-up
period of 18 months. Later, the patient had corneal perforation.
Her visual acuity showed light sense negative and recurrence
was noted on CT and bone scans. The patient underwent complete
tumor excision and total exenteration with implantation of
myocutaneous flap. After exenteration, no recurrence was noted
during 6 months follow-up.
Case 3
A 68-year-old man had a conjunctival mass over the left eye
for 1 month and decreased visual acuity for 1 year. Conjunctival
masses were noted over the nasal and temporal conjunctiva
and the superior and nasal cornea. A B scan revealed an intraocular
mass. CT revealed an inferior left orbital mass. Histopathlogic
examination results revealed well-differentiated SCC after
total exenteration was performed. One and a half months after
exenteration, the patient underwent augmentation of socket
with temporal myofascial flap and STSG for socket reconstruction.
After exenteration, no recurrence was noted during 20 months
follow-up.
Case 4
A 36-year-old woman had previously had intracranial meningioma
after surgery at a different hospital. Six years later, she
visited our hospital due to prominent proptosis for 6 months.
CT revealed left orbital tumor secondary to intracranial meningioma;
however, she refused treatment. When she was 49-year-old,
she was admitted due to weakness in her four limbs and prominent
proptosis. CT revealed a recurrent meningioma involving the
juxtasellar, suprasellar, and frontal areas. The patient underwent
combined surgery of right orbito-frontal craniotomy, tumor
excision, and eyelid- sparing exenteration, followed by right
rectus abdominis muscle flap by a neurosurgeon, plastic surgeon,
and ophthalmologist. Radiotherapy was performed, with the
total doses of 2880 cGy/16fr. Major depression was noted during
the radiotherapy; thus, the radiotherapy was suspended. The
patient was lost to follow up 1 year postoperatively.
Case 5
A 45-year-old man had complained of nasal obstruction and
purulent rhinorrhea for 10 years. During a visit at an otolaryngeal
clinic, a nasal mass was noted and CT revealed right ethmoid,
maxillary and nasal mass with posterior orbital wall defect.
The patient underwent right pansinusectomy. Histopathologic
examination results revealed the tumor as SCC. Radiotherapy
(6200 cGy/31fx) was performed. After 14 months, a right orbital
tumor with lateral rectus muscle and lateral wall destruction
was noted on magnetic resonance image (MRI) study. The patient
underwent right maxillectomy, total exenteration followed
by myocutaneous muscle flap and STSG. Chemotherapy was also
performed 6 months after exenteration. However, right orbit,
apex, and temporal base invasion were noted on a follow-up
image study 2 years after total exenteration. Fever, vomiting,
and poor appetite were noticed and patient died due to bacterial
meningitis.
Case 6
A 62-year-old woman complained of epistaxis for 1 week. Functional
endoscopic sinus surgery was performed in a different hospital
and malignancy was told, so she was referred to our hospital
for further treatment. At our otolaryngeal clinic, MRI was
arranged and revealed a mass in the maxillary, ethmoid sinus,
and optic nerve involvement , with brain spared. Thus, she
was referred to the ophthalmologic clinic for the combined
surgery. Her visual acuity was counting fingers 50-60 cm and
ocular ductions were completely limited in all directions.
An otolaryngologist and ophthalmologist performed the tumor
removal and total exenteration with spontaneous granulation.
Histopathological examination results of specimens included
rhabdomyosarcoma (alveolar type). Chemotherapy with oncovin,
endoxan, and adriamycin was given. Radiotherapy with the dose
of 6840 cGy/38fr was performed. Six months after beginning
chemotherapy, the patient came to the emergency room due to
abdominal distention and died due to suspected liver metastasis.
Case7
A 47-year-old man complained of blurred vision, eye pain,
and frequent discharge from the left eye. Tumor infiltrate
was noted near the superior and nasal conjunctiva and limbus.
CT revealed left lower orbital tumor. Aspiration cytology
from the infiltrate showed conjunctival SCC. Total exenteration
with spontaneous granulation was performed and the histopathological
examination results were well-differentiated SCC. Radiotherapy
was performed (4000 cGy/20fx). Postoperatively, socket skin
necrosis was noticed but no intracranial invasion occurred.
The patient died due to liver cirrhosis 10 years after total
exenteration (Table 1).
DISCUSSION
Exenteration is indicated in patients with malignant neoplasm
of the orbital contents, either primary or secendary extension
from adnexal tissue that cannot be controlled by simple excision
or radiotherapy.(2) It entails the removal of the eye together
with its extraocular muscles and the soft tissue of the orbit.(3)
According to the lesion excised, exenteration can be classified
into (1) total exenteration, (2) subtotal exenteration, and
(3) superexenteration.(4) Levin et al showed the following
indications for orbital exenteration: (1) eradication of presumed
life-threatening malignancy (89%), (2) eradication of life-threatening
infection (6%), and (3) alleviation of intractable pain or
deformity (5%).(3) Our study also revealed that orbital exenteration
was most frequently performed for the treatment of life-threatening
malignancies.
During the procedure of total exenteration, the incision site
was carried from the skin down to the level of periosteum.
The orbital contents with the periosteum were excised after
the periosteum was elevated from orbital bones. Subtotal exenteration
involved the removal of soft tissue with eyelid preserving.
Superexenteration required frontotemporal sphenoidal craniotomy
and en bloc orbitectomy. This procedure involved a neurosurgeon,
ophthalmologist, and plastic surgeon. In a study by Shields
et al, 16 exenterations were performed using an eyelid-sparing
technique and six were left to heal by spontaneous granulation.
They concluded that the eyelid-sparing technique was more
rapid healing and enabled earlier fitting of a prosthesis.
In performing an eyelid-sparing procedure, it is necessary
to leave some of the orbicularis muscle with the skin flaps,
which permits better vascularization.
The 0.3 mm skin graft was harvested from non-hair-bearing
anterior or inner sides of the upper thigh by the dermatome.
The exenterated orbit was well epithelialized within 3 to
4 months. During the surgery, the orbital content was cut
off using a snare. The snare itself was sometimes broken without
the contents removal if the instrument was not deeply inserted.
It was suggested that the snare should be tightened first
and then the whole instrument be rotated. Secondary orbital
tumor could arise from paranasal sinuses, nasopharynx, lacrimal
sac, CNS, skin (eyelid), and conjunctiva . The common presenting
symptoms included chronic sinusitis, epistaxis, nasal obstruction,
painful eye, nonaxial displacement of the globe, lid swelling,
diplopia, and blurred vision.(5)
Paranasal sinus tumors (SCC type) are the most common sinus
malignancies to invade the orbit and they most often involve
the maxillary sinus and then ethmoid sinus and frontal sinus.
Paranasal sinus tumors (Rhabdomyosarcoma type) involve the
ethmoid sinus the most. In addition, they are the most common
malignancies in head and neck of the children. They may originate
from the ethmoid sinus or nasal cavity and invade into the
orbit. They tend to involve the superior part of the orbit.
Rhabdo-myosarcoma is a highly malignant myogenic tumor and
is classified into four subtypes: pleomorphic (adult), embryonal,
alveolar, and botryoid. The alveolar type is the least common
of the subtypes.(6,7) The initial symptoms may include nasal
obstruction or epistaxis. Distant metastasis to the lung and
cervical lymph nodes has been reported. Surgical intervention,
chemotherapy, or radiotherapy should be combined for the treatment.
Secondary orbital tumors from rhabdomyosarcoma are rare and
patients usually have poor prognosis for long-term survival.
In a report of four patients by Walton et al, the most common
manifestations included proptosis, reduced visual acuity,
and motility disorders. Despite the combination of chemotherapy
and radiotherapy, all patients died within 6 months of orbital
metastasis.(7)
Conjunctival SCC arises from perilimbal conjunctiva. Clinically,
it presents as dysplasia, solar keratosis, and carcinoma in
situ. Because the tumors have low rates of recurrence, they
have been treated successfully with local resection and cryotherapy.
Eyelid SCC occurs commonly in fair-skinned elderly people
with a history of chronic sun exposure and skin damage.(8)
SCC, although less common than BCC, is more aggressive and
invasive. Complete surgical resection of the tumors has been
advocated due to the potential for metastasis and its lethality.(9,10)
The tumors must be treated using surgical excision and radiotherapy.
Eyelid BCC is the most frequent malignant lid lesion, representing
approximately 80% of eyelid neoplasms.(11-15) The malignant
cells arise from the so-called basal or germinal cells of
epidermis. Most lesions occur in fair-skinned individuals.
The lesions involve the lower lid (70%), medial canthal area,
upper lid, and lateral canthal area. BCC lesions do not metastasis,
and patients have shown 5-year tumor control rates of up to
95%. Cryotherapy for skin tumors has gained in popularity
due to the easy application and low rate of complications.(1,5,6)
The treatment involves a rapid freezing and a slow thawing
and creates a tissue temperature of -40 oC. Two to three cycles
follow each other. But I will treat patient as a combined
therapy of surgery and cryotherapy/irradiation, instead of
cryotherapy only. Radiotherapy may be useful for BCC, but
the results are not as satisfactory as those of cryotherapy.
Rhadomyosarcoma is the most common malignancy in head and
neck region of children.(16) Primary optic nerve meningioma
may arise from the optic nerve sheath and meningeal cell structures
near the optic nerve. Orbital involvement by intracranial
meningioma is rare and arises from the sphenoid bone. Treatment
of intraorbital meningioma is controversial. Visual improvement
is not worth the risk of surgery.(17,18) Radiotherapy plays
little role in the treatment.(19,20)
Conclusion
Secondary orbital tumors may arise from the sinus, pharynx,
meninges, brain, eye, conjunctiva, eyelid, and lacrimal sac.
Therefore, the imaging studies must encompass the fields of
the orbit, sinus, and brain.
Total exenteration with spontaneous granulation is more effective,
more acceptable for cosmesis, and easier for detection of
recurrence than other therapies. However, it is time-consuming
for complete granulation (3-4 months). Total exenteration
with lining of orbital walls with temporalis muscle transplantation
and STSG has been suggested by many surgeons.(21,22) After
this procedure, the wound heals rapidly and it is easy to
care for. However, it is cosmetically unacceptable due to
the over-sized graft and it is difficult to detect the recurrence.
Subtotal exenteration is also effective in saving lives, more
rapid healing (1-6 weeks) and enables earlier fitting of prosthesis
than other treatment modalities. During the procedure, it
is important to leave some orbicularis muscle with the skin
flaps to permit continued vascularization, and to prevent
them from becoming necrotic.(23,24)
Total/subtotal exenteration with spontaneous healing for the
sake of early detection of recurrence and good cosmesis is
advocated. Delayed socket reconstruction with temporalis muscle
rotational flap and STSG is also indicated if poor healing
of apex occurs.
Acknowledgments
We appreciate the combined efforts of the plastic surgeons,
neurosurgeons, and otolaryngologists to complete the operations.
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