Orbitocranial Approach for Adenoid Cystic Carcinoma of the Lacrimal
Gland Reconstructed with an Osteocutaneous Free Flap: A Novel Technique
Akshay Sanan MD1, Greg Smith MD1, Jurij R. Bilyk MD2, Ryan N. Heffelfinger MD1
of Otolaryngology-Head and Neck Surgery, Thomas Jefferson University Hospital, Philadelphia, PA;
2Skull Base Division, Neuro-Ophthalmology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, PA
CASE REPORT, CONTINUED
SURGICAL TECHNIQUE, CONTINUED
Educational Objective: At the end of this presentation, participants
should understand the reconstructive options for superior & lateral bony
loss with orbital exenteration. We report a novel reconstructive modality
with an osteocutaneous free flap for a resection done via an orbitocranial
approach for locally invasive adenoid cystic carcinoma (ACC). We
describe the strategy and technical details of operative maneuvers to
maximize the potential for successful future orbital prosthesis.
Study Design: Case report and literature review.
Methods: The case of a 20 year old woman who presented to a tertiary
care academic medical center with a recurrent ACC of the right lacrimal
gland is reported and reviewed. The patient’s pertinent history, clinical
findings and radiologic studies are examined. Figures and intraoperative
photography will demonstrate surgical specifics.
Results: The patient underwent resection via orbitocranial approach.
Resection included orbital exenteration and resection of the superior and
lateral orbital rim on the right side. A radial forearm osteocutaneous free
flap was used for reconstruction. The bone of the forearm provided
anatomical replacement of the orbital rims, while the thin
fasciocutaneous soft tissue allowed for a concave reconstruction of the
orbital cavity. Brow position was maintained. The patient’s post-operative
course was unremarkable.
Conclusion: This is the first report of osteocutaneous free flap
reconstruction for defect from ACC resection via orbitocranial approach.
Prior studies have reported orbitocranial approach reconstruction with
fasciocutaneous free flaps. Orbital bone removal for adequate margins
and/or oncologic resection is a routine part of tumor resection for these
malignancies. The osteocutaneous free flap allows for better aesthetic
results and affords reconstruction of the normal orbital rim contours.
The bone of the forearm provided anatomical replacement of the orbital rims, while the thin
fasciocutaneous soft tissue allowed for a concave reconstruction of the orbital cavity.
Post-operatively, the patient underwent adjuvant radiotherapy. Sequelae of this yielded loss of the right eye
brow hair (Fig. 2, 3). The patient underwent debulking and molding of the free flap to improve contour (Fig.
3). She is currently being evaluated and fit for an orbital prosthesis.
The orbital roof was opened towards the dura. Bone cuts were made in the
area of the frontozygomatic suture across the superior orbital rim and
extending medially to the frontal sinus and posteriorly to the orbital apex. The
frontal sinus and nasofrontal outflow region mucosa was cauterized and
removed. The inferior osteotomy cut was made extending from the frontal
sinus down through the medial aspect of the superior orbital rim and towards
the ethmoid sinuses. A 2-cm margin circumferential to the area of gross tumor
involvement was taken. (Fig. 4A) The orbital roof was removed to the level of
the superior orbital fissure. The underlying dura appeared completely intact and
was preserved. A sheet of bovine pericardium and titanium mesh was fashioned
on the orbital side of the missing roof to minimize any postoperative pulsations
of the frontal lobe against the flap. The mesh was placed without evidence of
CSF leakage and is seen in the post-operative images (Fig. 6).
Reconstruction was then performed with an osteocutaneous radial forearm
free flap from the non-dominant arm. A thermoplastic splint was used to
estimate the optimal periorbital contour and plan the size of the bony segments
(Fig. 4B), which were used to replace the missing lateral orbital rim and frontal
bar. A single osteotomy provided the directional need of the bones. The skin
component of the flap was folded upon itself to form a cone, and used to
resurface the bony reconstruction, ensuring a concave contour. A penrose drain
was laid on the orbital floor and exited the inferior aspect of the skin incision.
Another thermoplastic splint was used to maintain the concavity of the orbit.
This was sutured to the surrounding facial skin and left in place for 2 weeks.
After postoperative radiation therapy, we performed a revision procedure to
fine-tune the orbital contour in preparation for an orbital prosthesis.
Adenoid cystic carcinoma (ACC) of the lacrimal gland is a rare
epithelial orbital disease. However, it is the most common epithelial
malignancy of the orbit. Controversy exists over the optimal
management of this disease. Some authors advocate conservative eye
sparing surgery with adjuvant external beam radiation, proton beam
radiation, or intra-arterial chemotherapy via the lacrimal artery. Others
believe that radical orbital exenteration with or without removal of
surrounding bone for margins is appropriate. There is also no
consensus on reconstruction of the orbital defect.
We present a case of recurrent ACC of the lacrimal gland treated
with radical exenteration. This approach is designed to obtain adequate
margins with the intent of decreasing local and regional recurrence. The
novelty of this case was reconstruction of the bony orbit with an
osteocutaneous free flap. The purpose was to describe a means for
orbital prosthesis to achieve improved aesthetic outcomes.
Figure 1: Preoperative images; Figure 2: 2 months post-operative; Figure 3: After flap debulking
Figures 4: A. Intra-operative images detailing resection. B. Aquaplast mold provided free flap contour.
A 20 year old female presented to our institution for consultation
regarding ACC of the right lacrimal gland. She was treated nine years
prior with brachytherapy. Recently, she developed a dull brow ache over
several months duration. Fullness in the upper right eyelid prompted a
biopsy, which revealed ACC. The patient was referred to our clinic for
evaluation of disease resection and reconstruction of the frontal bone
The patient had no other significant medical history. Her prior
surgical history included wisdom teeth removal. There was no family
history of ACC. The patient denied smoking or drinking.
The patient’s exam was notable for some mild limitation of her
upgaze. Facial sensation was diminished in the right V1 distribution,
more so to touch than to pinprick. The patient had normal and
symmetric facial movements and hearing. The remainder of her cranial
nerve exam was normal. Pupils were equal, round, and reactive and
visual fields were full to confrontation bilaterally.
Figure 1 shows the patient pre-operatively. Pre-operative CT and
MRI of the orbits (Fig. 5A-C) revealed a lobulated, homogeneously
enhancing mass in the region of the right lacrimal gland. The mass
demonstrated heterogeneous T2 and isointense T1 signal. There was
remodeling of the right orbital roof, including thinning of the right
anterior orbital rim. There was no evidence of intracranial extension.
The patient was taken to the operating room for radical exenteration.
Skull base resection extended to the frontal dura (Fig. 4A). The
eyebrow was spared during the resection. Reconstruction was achieved
with an osteocutaneous radial forearm free flap.
ACC of the lacrimal disease is a rare entity and the literature includes case
reports and small case series. The treatment and reconstructive methodologies
vary. The goal of this paper is to describe a novel reconstructive technique.
Many clinicians perform globe-sparing procedures or orbital exenteration
alone for these tumors and remove surrounding bone only when it appears
clinically or radiographically involved. When superior and lateral orbital bones
are removed for surgical margins, we advocate reconstruction with an
osteocutaneous flap to improve postoperative cosmesis. Prior studies have
employed fasciocutaneous free flap reconstruction for the orbitocranial defect.
Bony orbital rim has been reconstructed with split-calvarial bone grafts.
Goals of reconstruction of the bony orbit are multifactorial. If the patient
hopes to have an orbital prosthesis, the surgeon must have certain
reconstructive objectives. Prosthetics can be placed either in an “open” cavity
where a concave orbital socket is created or in a “closed” cavity where the orbit
is filled with soft tissue to the level of the orbital rim. One of the drawbacks of
“open” reconstruction is potential displacement of the brow and cheek because
of soft-tissue contracture. It is advised to over-correct the reconstruction to
better accommodate soft tissue changes from wound healing and potential
adjuvant radiotherapy. Revisions of the free flap can be performed and include
flap debulking by direct tissue excision in addition to suction assisted lipectomy.
A concave orbital cavity is optimal for an orbital prosthesis. If there is excessive
tissue bulk, the prosthesis fits poorly and is not usable.
Placement of an osseous component of a free tissue transfer allows for
significant functional and aesthetic improvement of the orbital subunit. Free
vascularized bone also resorbs less than bone grafts. The goal of the osseous
component is to allow for normal bony contour once soft tissue swelling
subsides. The bony contour also allows for optimal brow position, which is
essential for a natural appearance.
Although we present a single case with limited follow up, our overall
purpose was to describe a novel periorbital reconstructive method in a young,
high “aesthetic demand” patient. Our ultimate goals were to allow for orbital
prosthesis placement and maximize aesthetic outcome.
This is the first report of an osteocutaneous free flap reconstruction of a
periorbital defect from ACC resection via orbitocranial approach. Prior studies
have reported periorbital reconstruction with fascio-cutaneous flaps and free
bone grafts. Orbital bone removal for adequate margins and oncologic resection
is a routine part of tumor resection for these malignancies. The osteocutaneous
free flap allows for better aesthetic results, reconstruction of the normal orbital
rim contours and orbital prosthesis placement.
Figures 5: A. Pre-operative T1 MRI with isointense signal and remodeling of orbital roof, B. CT
demonstrating bone remodeling, C. 3D reconstruction.
Figures 6: A. Post-operative MRI, B. CT, C. 3D reconstruction.
Note bony orbital reconstruction with radius.
The goal of orbitocranial resection is to achieve en bloc removal of orbital contents, tumor, and adjacent
bony structures to ensure adequate margins.
An incision was made circumferentially around the orbit, sparing the eye brow. A soft tissue orbital
exenteration was performed to the level of the optic canal and superior orbital fissure. The specimen was
removed en bloc. The skull base resection started with an osteotomy to the right orbital roof and floor of the
anterior cranial base down to the dura, staying posterior to the gross anterior cranial base tumor involvement.
Esmaeli B et al. Surgical management of locally advanced adenoid cystic carcinoma
of the lacrimal gland. Ophthal Plast Reconstr Surg 2006;22:366-70
Wilson KF et al. Orbitocranial Approach for Treatment of Adenoid Cystic
Carcinoma of the Lacrimal Gland. Annals of Otology, Rhinology & Laryngology
Esmaeli B, et al. Outcomes in patients with adenoid cystic carcinoma of the lacrimal
gland. Ophthal Plast Reconstr Surg 2004;20:22-6.
Polito E, Leccisotti A. Epithelial malignancies of the lacrimal gland: survival rates
after extensive and conservative therapy. Ann Ophthalmol 1993;25:422-6
Qin W et al. Adenoid cystic carcinoma of the lacrimal gland: CT and MRI findings.
Eur J Ophthalmol 2012; 22(3): 316-9
Chepeha DB et al. Restoration of the orbital aesthetic subunit in complex midface
defects. Laryngoscope. 2004;114:1706-1713
Hanasono et al. An algorithmic approach to reconstructive surgery and prosthetic
rehabilitation after orbital exenteration. Plast Reconst Surg. 2009;123(1):98-105