Case Report
Cryo Assisted Minimally Invasive Surgery for the
Treatment of Orbital Cavernous Haemangiomas
Muhammad Amer Yaqub, Saadullah Ahmad, Muhammad Khizar Niazi,
Teyyeb Azeem Janjua,
Omer Farooq
Pak J Ophthalmol 2013, Vol. 29 No.
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See
end of article for authors
affiliations
..
.. Correspondence
to: Muhammad Amer Yaqub Classified Eye Specialist Oculoplastic
Surgeon AFIO Rawalpindi,
..
.. |
Purpose: To study cryo assisted minimally invasive surgery for the
treatment of orbital cavernous hemangiomas. Material and Methods: We present a case series of six patients having orbital
cavernous haemangiomas. All patients underwent
minimally invasive cryosurgical extraction of the tumour at the Armed Forces
institute of Ophthalmology, Rawalpindi. Results: All the
tumours were successfully removed en bloc with the help of cryo extraction.
The vision was improved in all but one case. The recovery was uneventful and
histopathological reports confirmed the diagnosis after more follow-ups of
nine months, no recurrence was observed in any of the operated eye. Conclusion: Cryo-assisted minimally invasive surgery offers an exciting
approach for management orbital cavernous hemangiomas, with good cosmetic
results and early functional recovery. |
Cavernous haemangioma is the most common primary benign vascular
orbital tumour.1 It presents with painless unilateral proptosis that is
reducible and associated with hyperopia.2 Most cavernous
haemangiomas are intraconal and lateral in location. They result from new
vessel formation, proliferation of tissue components of the vessel wall, and
hyperplasia of cellular elements of vascular origin.3 Computed
tomography (CT) and magnetic resonance imaging (MRI) are of particular
importance in the diagnosis of orbital vascular lesions.
Surgical access to posterior orbit is difficult because of
the presence of delicate structures including optic nerve, ophthalmic artery
and veins, extra-ocular muscles and nerves. Surgical approaches to treat
orbital disease should provide a good exposure of intra-orbital anatomical
structures, and provide good cosmetic results. Different approaches to the
intra-orbital space have been described in the literature.4
In
an attempt to maintain this philosophy and to avoid large incisions still
providing an increased operative exposure, we describe cryo-assisted minimally
invasive approach which mostly avoids bone removal or intraconal muscle
sectioning. It provides access to the superior, medial, lateral, and inferior
quadrants of the orbit, depending on the extent of the conjunctival and eyebrow
incisions. Thus, complete exposure and resection of very large intra-orbital
lesions is feasible with reduced morbidity.
MATERIAL AND
METHODS
We
describe the outcome of 6 patients with cavernous haemangioma of the orbit
treated in our department between April 2008 and Feb 2010. Study Design was
interventional case series. Patients underwent minimally invasive surgery
through different approaches, completely removing the angiomas. All patients
with intraconal mass and a strong clinical suspicion of cavernous hemangiomas
underwent minimally invasive cryosurgical extraction. Patients having any
surgical intervention in the past were excluded from the study. The presenting
features of all six cases in given in table-1. Pre-op marking of site of
incision and tumor was carried out. The tumor was reached either by lateral
orbitotomy, a lid crease incision, or via trans- conjunctival approach
depending upon location of tumor (table 2). After securing the vascular
connections, lesion was extracted en bloc with the help of cryo-probe and blunt
dissection. Wound was closed in layers. Histopathology was carried out in the
end to confirm the diagnosis.
RESULTS
The mean age of patients was 32 years with a range of 16- 55
years. All the tumours were successfully removed en bloc with the help cryo
extraction. The vision improved in all but one cases, and table-3 shows the
comparison of pre-op and post-op visual acuity of the selected cases. The
recovery was uneventful and histopathological reports confirmed the diagnosis
of cavernous hemangioma. After a mean follow-up of nine months, no recurrence
was observed in any of the operated eyes.
CASE 1
A 32 year old female presented with a 07 years history of
painless progressive proptosis and impaired visual acuity of her right eye. She
denied history of trauma. In the past, she was given a course of systemic
steroids and biopsy was attempted elsewhere. Her systemic and medical history
was not contributory. She was admitted in another institute where she was given
a course of systemic steroids and biopsy was taken which was non diagnostic
because the sample was inadequate. She was using only lubricants on admission
to our hospital. On examination the best corrected visual acuity (BCVA) was
6/36 in right eye and 6/6 in left eye. Right eye showed proptosis, diplopia in
primary gaze, chemosis and restriction of extraocular movements in all gaze
positions. Examination of right fundus showed choroidal folds. CT scan orbit
and brain with coronal sections showed a well circumscribed intraconal lesion
in right orbit. A plan for excisional biopsy was made.
The mass in her right orbit was approached via a subciliary
incision. Extensive adhesions were encountered after opening the orbital
septum, probably from her previous biopsy. These were lysed and the rest of the
mass was extracted en masse using a cryoprobe and blunt dissection. The
recovery was uneventful. Histopathology revealed an encapsulated cavernous
haemangioma. Her vision improved to 6/9, the choroidal folds resolved and
ocular movements recovered in all gaze positions (Fig. 1 and 2).
Fig. 1: 7th
Post-op Day in OPD in primary position.
Fig.
2: Subciliary incision sutured with 7/0
Vicryl with mild lower lid post op. edema.
CASE 2
A 37 years old man presented with gradual painless proptosis
with gradual loss of vision in his left eye for the last 08 years. BCVA in
right eye was 6/6, while in the left eye was 6/60. Left eye showed axial
proptosis of 35 mm and supero-temporal dystopia of 5 mm (Fig. 3). Extra-ocular
movements were restricted in all gaze positions. There was no retropulsion or
bruit. Fundus showed choroidal folds. Examination of right eye was unremarkable.
CT scan showed a large well circumscribed intraconal lesion in left orbit (Fig.
4).
Fig. 3: Proptosis and suprotemporal dystopia of left eye.
Fig. 4: CT scan axial view showing large well circumscribed intraconal
lesion in left orbit.
Fig. 5: Three weeks after the surgery showing resolved proptosis
and dystopia.
The
patient was planned for orbitotomy under general anesthesia. After marking an
extended lazy S incision till zygomatic arch, lateral orbital margin was
removed and the lesion approached through blunt dissection. On opening the
periorbita, a plump, nodular, encapsulated mass with vascular channels on its
well defined surfaces was found. An apical vascular tag was identified and
cauterized; to prevent the gush of blood. A cryoprobe assisted extraction was
done. The lesion was completely excised. Wound was closed in layers. Visual
acuity of patient was checked in the evening to exclude optic nerve
compression. Histopathology confirmed the diagnosis of multi-lobulated, well
encapsulated left cavernous haeman-gioma. Vision in the left eye improved to
6/24 six weeks after the surgery (Fig. 5).
Fig. 6: Right eye with inferior dystopia and proptosis.
Fig. 7: CT Scan revealed a large extraconal, well
circumscribed lesion in supero-nasal region of right orbit.
CASE 3
A 35 years old female presented to our
hospital with inferior displacement of right eye for last 04 years associated
with gradual deterioration of vision. There was no history of trauma or any
systemic illness. On ocular examination BCVA in right eye was 6/36 while that
in left eye was 6/6. Right eye showed 6 mm inferior dystopia, 2 mm of proptosis
compared to left eye and restriction of ocular motility more marked in vertical
than in horizontal gaze (Fig. 6). There was no retropulsion or bruit. Fundus
showed choroidal folds. Examination of left eye was unremarkable. CT-Scan
revealed a large extraconal, well circumscribed lesion in supero-nasal region
of orbit (Fig. 7).
Patient
was planned for orbitotomy under general anesthesia. Pre op. marking was made
(Fig. 8). Lid crease incision extending temporally 15 mm lateral to temporal
orbital margin was made. After penetrating the septum, the lesion was
approached through blunt dissection which revealed a well encapsulated nodular
mass. Vascular connections were secured
and the lesion was removed en bloc with the help of cryoprobe (Fig. 9). It
measured 35 x 22 mm. Wound was closed in layers. Histopathology confirmed the
diagnosis of multi-lobulated, well encapsulated cavernous hemangioma. Vision in
the left eye improved to 6/12 two weeks after surgery.
Fig. 8: Right upper lid crease marking extending temporally.
Fig. 9: Lesion being removed en bloc with the help of
cryoprobe.
CASE 4
A 16 years old male presented to our
hospital with inferior displacement of right eye for last 3 years associated
with gradual deterioration of vision (Fig. 10). There was no history of trauma
or any systemic illness. On ocular examination BCVA in right eye was 5/60 while
visual acuity in left eye was 6/6. Right eye showed 07 mm inferior dystopia, 04
mm of proptosis compared to left eye and restriction of ocular motility more
marked in vertical than in horizontal gaze. There was no retropulsion or bruit.
Fundus showed choroidal folds. Examination of left eye was unremarkable. CT Scan
revealed a large extraconal, well circumscribed and encapsulated lesion in
supero-nasal region of orbit.
Patient
was planned for orbitotomy under general anesthesia. Lid crease incision
extending temporally 13 mm lateral to temporal orbital margin was made. The
lesion was reached through blunt dissection. The tumour was found to be plum
coloured well encapsulated nodular mass. After securing the vascular
connections, lesion was extracted en bloc with the help of cryoprobe. It
measured 30 X 20 mm. Wound was closed in layers. Histopathology
confirmed the diagnosis of multi-lobulated, well encapsulated left cavernous
hemangioma. BCVA in the right eye improved to 6/12 six weeks after the surgery
(Fig. 10).
Fig. 10: Inferior dystopia and proptosis of right eye.
CASE 5
A 65
years old man presented with gradually enlarging mass below right eye for last
3 years. On examination visual acuity in right eye was hand movement (HM)
positive. He had relative afferent pupillary defect in the same eye.
Extraocular
Fig. 11: Six
weeks after the surgery.
Fig. 12: Mass bulging through inferior fornix causing mechanical
ectropion.
Fig. 13: Well demarcated
homogenous hyper dense mass involving inferonasal aspect of right orbit.
Fig. 14: Lesion below right eye ball visible under conjunctiva and with
bluish discoloration of lower lid.
Fig. 15: Brownish well encapsulated mass measuring 3.0 X
2.8 cm was extracted en bloc.
Fig. 16: Cosmetic improvement three weeks post operatively.
Table
3: Decimal Equivalent of
Pre-op ad Post-op visual acuities of cases (n=6)
movements
were severely restricted. Mass was visible bulging through inferior fornix
causing mechanical ectropion. (Fig. 12) CT scan showed a well demarcated homogenous hyper dense
mass involving infero-nasal aspect of right orbit (Fig. 13). Cryo-assisted
extraction through trans-conjunctival approach was done. Patient made smooth
post operative recovery but his vision did not improve.
CASE 6
A 30
years old female presented with gradual deterioration of vision and swelling
below right eye ball for last one and half year. (Fig. 14) On examination her
BCVA was 6/24 in right eye and 6/6 in left eye. There was a mass involving
inferior aspect of right orbit but not causing any dystopia. Growth was visible
under the conjunctiva in inferior fornix. Lesion was excised through
trans-conjunctival approach. A brownish well encapsulated mass measuring 3.0 X
2.8 cm was extracted en bloc. (Fig. 15) Post operative recovery was uneventful
and patients visual acuity improved to 6/9 three weeks post operatively (Fig.
16).
DISCUSSION
Cavernous haemangiomas are the most common benign,
non-infiltrative neoplasms of the orbit and represents 9.5 to 15% of the
primary expansive lesions of the orbit.5 They usually grow slowly between the
extrinsic muscles and present as a mass effect on the globe.
It is a vascular malformation characterized by the presence of sinusoids with
fine walls, which contain an accumulation of blood with no apparent arterial or
venous inflow.7 Its incidence peaks between the ages of 40 50
years and women are affected more.8
A slowly progressive painless proptosis
is the typical presenting symptom. Impaired extraocular movements and visual
function are seen with large lesions and with lesions located at the orbital
apex.9 Most cavernous haemangiomas are located
within the intraconal space. Nearly
all patients with cavernous
haemangioma can be correctly diagnosed
by preoperative radiological studies.10 Orbital CT
scan is the single most useful diagnostic test and shows a well circumscribed
lesion with no osseous involvement.2 In our study, CT scan showed
very well defined smooth mass, which enhanced with intravenous contrast.
Surgical treatment is recommended for optic nerve
compression as evidenced by visual field defects, optic nerve swelling and
pallor. Other indications include diplopia and bothersome cosmesis.11
Several therapeutic modalities and surgical approaches have been described, in
order to preserve the normal orbital structures. Most approaches used to remove
orbital tumours typically include bone removal (orbitotomies) with or without
craniotomies.12 Complete excision is
generally accomplished without recurrencesas the tumour is well encapsulated with relatively
few feeding vessels.13,14 Lateral orbitotomy has been widely employed for the removal
of orbital tumors, being used in large sized hemangiomas, especially those
located in the lateral compartment of the orbit and orbital apex.15
Transconjunctival approach can be used successfully for
anterior as well as retrobulbar intraconal cavernous hemangiomas.16
Anterior orbitotomy is useful in many cases, without significant complications
and warrants more favourable consideration if combined with the use of
cryoprobe and surgical microscope.17 Postero-inferior orbitotomy
through the maxillary sinus18 can be used in small, well demarcated
lesions in the posterior and inferior orbit near the apex. Endoscopic
trans-ethmoidal approach of the orbit is a minimally invasive surgery for
retro-bulbar orbital neoplasm, leading to excellent cosmetic results with less
bleeding. The medial wall of the orbit, the orbital apex, and the optic canal
can be exposed through a middle meatal antrostomy, an anterior and posterior
ethmoidectomy, and a sphenoidotomy.19 Lateral suprabrow,20
trans-nasal21 and the combined pterional and orbitozygomatic
approach22 are employed for removal of tumors affecting the optic
canal.
Transcranial approaches offer an excellent surgical exposure
and a good cosmetic outcome and should be considered for big lesions located
superiorly or medially to the optic nerve, especially those involving the apex.23
Complications of surgery include ptosis, impairment of ocular movements,
diplopia due to mechanical or vascular trauma and visual disturbances.10
Careful preoperative workup including plan for minimal
invasive approach based on CT scan offered excellent exposure and
a rewarding cosmetic result in our cases and that this technique might
also be considered for larger lesions Figure 1 4 show pre-and post-operative
picture of one of cases with technique of en bloc removal of these tumours. Avoiding large incisions and osteotomies resulted in early
rehabilitation and decreased morbidity.
Additionally, there were no risks of cerebrospinal fluid leakage or
significant blood loss during the procedure, no bone removal or reconstruction
was required and no postoperative enophthalmos or temporal muscle atrophy was
encountered.
CONCLUSION
Cryo-assisted
minimally invasive surgical excision offers an exciting approach for management
orbital cavernous hemangiomas. With improvement of neuroimaging techniques, it
is possible to obtain a correct pre-operative diagnosis in almost every case of
vascular orbital lesions. This can lead to excellent cosmetic results with
complete functional recovery and minimal morbidity.
Authors Affiliation
Dr. Muhammad Amer Yaqub
Classified Eye Specialist
Oculoplastic Surgeon
AFIO
Rawalpindi
Dr. Saadullah Ahmad
Graded Eye Specialist
CMH
Malir
Dr. Muhammad Khizar Niazi
Classified Eye Specialist
Vitreoretinal Surgeon
AFIO
Rawalpindi
Dr. Teyyeb Azeem Janjua
Graded Eye Specialist
CMH
Bannu
Dr. Omer Farooq
Graded Eye Specialist
PNS Shifa, Rawalpindi
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