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Extended orbital exenteration is a highly disfiguring operation which entails complete removal of the orbital contents including periorbita, eyelids and involved surrounding bony walls with variations tailored to the specific clinical circumstances. The aim of such an extensive surgery is to achieve local control of the life-threatening progressive neoplasms, when other treatment modalities fail to achieve disease control. Eyelids can be preserved in posterior orbital pathology, while it may not be possible in neoplasms arising from the anterior eye tissues. Depending on the clinical circumstances, if the neoplasm is invading the surrounding bony orbit, the involved bony and soft tissue structures are removed en bloc to achieve complete resection (R0 resection). Although the steps of the orbital exenteration are well defined, the same is not true for extended orbital exenteration. We demonstrate the details of extended orbital exenteration in different clinical scenarios for the malignancy of orbit and periorbital tissues invading surrounding orbital walls.
Department of Trauma Surgery, All India Institute of Medical Sciences, India
Novfa Iftikhar
Department of Internal Medicine, Government Medical College, India
Usaamah Javaid
Department of Internal Medicine, Shadan Institute of Medical Sciences, India
*Address all correspondence to: drahhakim@gmail.com
1. Introduction
Any ocular or periocular tumor, if neglected, can invade the orbit and raise the probability of various forms of orbital exenteration. Approximately 2–4% of the periocular malignancies invade the orbit and are candidates for orbital exenteration or extended procedures [1, 2, 3, 4, 5, 6]. Frezzoti et al. classified orbital exenterations into subtotal, total and radical (Table 1) [7]. The radical resections or extended orbital exenteration have been classified as Type V (removal of bony walls) and VI (removal of bony walls and adjacent structures) (Table 1) [7]. Exenteration or its variations are psychologically and anatomically disfiguring, hence reserved to treat potentially life-threatening malignancies unresponsive to other treatments. Extended orbital exenteration has wide variations to the basic technique and is tailored to the clinical circumstances. These variations depend on saving or sacrificing different tissues within or around the orbit. Clinical and radiological findings guide the surgeon to tailor make the resection needed to completely remove the tumor with negative margins along with the selected sections of the orbital bone. Extended orbital exenteration, although highly disfiguring surgery, offers the best chance of cure as it aims to achieve local control of extensive disease when other treatment modalities fail to halt the progression of the disease [8]. The basic technique is somewhat similar to the orbital exenteration which removes all orbital contents including the periorbita along with part or complete eyelids. But variations are tailor made depending on the extent of the disease as assessed by the CT or MRI scan [9, 10]. Eyelids may be spared if the pathology present posteriorly not infiltrating them [11]. We describe different indications and step by step approach to different clinical presentations of orbital tumors.
Stage
Type
Surgical technique
Subtotal exenteration
I
Eyelids and palpebral and bulbar conjunctiva sparing
II
Eyelids and palpebral conjunctiva sparing
III
Eyelid skin and deeper muscle layer sparing
Total exenteration
IV
Eyelid resection
Radical exenteration
V
Resection of orbital cavity bones
VI
Extension of adjacent structures
Table 1.
Categorization of orbital exenteration based upon surgical technique.
Any tumor arising from the globe or periocular tissues with involvement of orbital apex, full thickness periorbita or periosteum, retro-orbital fat, extraocular muscles, conjunctiva and sclera form an indication for orbital exenteration (Table 2). Different Tumors which result in orbital invasion are depicted in Table 3.
Indications of orbital exenteration and extended procedures
Primary tumors of globe with Malignant tumors arising from eyelids and surrounding skin with. Orbital sarcomas with. Lacrimal gland tumors with. Tumors of the paranasal sinuses and nose with. Lacrimal sac tumors
I
Orbital apex
II
Invasion through periorbita
III
Involvement of retro-orbital fat
IV
Extension into the extraocular muscles
V
Invasion of the conjunctiva or sclera
VI
Extension of adjacent structures
Table 2.
Indications for orbital exenterations.
Different pathologies involving the orbit and peri-orbital tissues
Periocular cutaneous malignant tumors
Basal cell carcinoma
Squamous cell carcinoma
Sebaceous gland carcinoma
Conjunctival tumors
Ocular surface squamous cell carcinoma
Mucoepidermoid variant
Spindle cell variant
Malignant melanoma
Choroidal melanoma
Choroidal melanoma with extra-scleral extension
Sarcomas
Rhabdomyosarcoma post chemoradiation residual/recurrent
Sino-orbital aspergillosis involving retrobulbar tissues and the orbital apex
Sino orbital Mucormycosis involving retroorbital tissue and the orbital apex
Table 3.
Common pathologies of eye and orbit.
2.1 Malignant tumors arising from eyelids and surrounding skin
Basal cell carcinoma is the most common malignant skin tumor accounting for 90% these cases; squamous cell carcinoma and sebaceous gland carcinoma each comprising approximately 4–6% of cases [1, 2, 3, 4, 5, 6, 8, 9, 10, 11, 12, 13]. The reported incidence of orbital invasion in basal cell carcinoma is 1.6–2.5% and risk factors include multiple recurrences, large size, aggressive histological subtype like infiltrative and morpheic patterns, perineural spread, canthal location particularly the medial canthus and age over 70. The incidence of orbital invasion is high in squamous cell 5.9% and much higher in sebaceous gland carcinoma 6–45% [14, 15].
2.2 Malignant conjunctival tumors
Advanced and invasive conjunctival melanoma and ocular surface squamous cell carcinoma require orbital exenteration. Approximately 15% of ocular surface squamous cell carcinoma invade orbit as reported McKelvie et al. [16]. The rare variants of ocular surface carcinoma like mucoepidermoid or spindle cell carcinomas and are better controlled orbital exenteration [11].
2.3 Orbital sarcomas
Orbital rhabdomyosarcoma is presently treated with combination of radiotherapy and chemotherapy. Orbital exenteration may be indicated either in poor responders or recurrent sarcomas. Alveolar soft part sarcomas may need upfront orbital exenteration with adjuvant radiotherapy.
2.4 Lacrimal gland tumors
Advanced adenoid cystic carcinoma of the lacrimal gland especially stage III and IV is an indication for orbital exenteration. Adenoid cystic carcinoma of the lacrimal gland is known to invade bone as well as demonstrate perineural invasion.
2.5 Tumors of the paranasal sinuses and nose
Paranasal sinuses and nose tumors may also require exenteration in case they extend to the orbital apex, complete thickness invasion through periorbita into posterior orbital fat, extension into the extraocular muscles and invasion of the bulbar conjunctiva or sclera.
2.6 Lacrimal sac tumors
Invasive neoplasms arising lacrimal sac like squamous cell, adenoid cystic carcinoma with infiltration into the orbit is an indication for orbital exenteration.
2.7 Invasive fungal infection
Uncontrolled invasive fungal infection like invasive aspergillosis or Mucor mycosis may need orbital exenteration for control.
We will describe step by step approach of various forms of orbital exenterations. The basic procedure is lid sparing total orbital exenteration and other forms are modifications of this procedure. All orbital contents are removed in entirety with preservation of the major part of the upper and lower lids. Once again we are stressing the fact that any form orbital exenteration is a radical procedure with high degree of disfigurement and therefore should be only considered when there is a valid indication as mentioned above.
Lid preserving orbital exenteration is an excellent surgical procedure as it takes care of the challenge of providing a good concave stable skin cover over which customized prosthesis can be glued. If it is not oncologically safe to preserve eyelids, they can be sacrificed and orbital cavity can be lined by temporalis fascia/muscle, fore head flap or split-thickness skin graft. Some surgeons do advocate spontaneous granulation method, however healing by granulation takes a long time and needs intensive post-operative care. Lid sparing technique avoid these sequels provided this method is technically feasible. This method, popularized by Coston and Small, is the modification of total exenteration technique which preserve major parts of both the eye lids [17]. A transverse blepharorrhaphy is done to cover the orbit thus ensuing good cosmesis and fast healing. This method also spares orbicularis muscle which provides an excellent vascular supply to the skin flap enhancing early healing.
Orbital exenteration should be performed under general anesthesia. Incision is marked on closed eyelids approximately 2 mm beyond the eyelash line including medial and lateral canthus (Figure 1). A sterile gauze piece is placed in the cul de sac in conjunctival tumors to avoid maceration. The eyelid can be divided into two lamellae, namely, the anterior and the posterior. The anterior lamella consists of the skin and orbicularis muscle, while the posterior lamella is formed by tarsus and conjunctiva. Three traction sutures with 4–0 silk are placed through the upper and lower tarsi to provide traction on the orbital contents (Figure 2A and B). Incision is then made with radiofrequency probe, 15 no blade or cutting diathermy along the skin markings (Figure 3A and B). Further dissection is done in pre-septal plane, which avoids injury to orbital septum, especially important in the cases where tumor is present in the anterior orbit. This technique spared orbicularis muscle which provides an excellent vascular supply to the skin flap. Dissection is further continued with bipolar cautery till the orbital rim is reached (Figure 4A and B). The periosteum along the arcus marginalis or orbital rim is incised 3600 (Figure 5A and B). Periosteal elevator is used to dissect the periosteum off the orbital rim and continued all the way to the orbital apex (Figure 6). Medially, along the superior orbital rim, superior orbital notch is encountered. The supra orbital neurovascular bundle is identified and cauterized (Figure 7A and B). On the medial wall, subperiosteal dissection is done from the anterior to the posterior lacrimal crest remaining medial to the lacrimal sac. Meticulous dissection is required here to avoid fracture of the thin lamella papyracea. Mostly periosteum can be easily elevated as it is loosely adherent to orbital bones, except in certain locations like sutures and fissures where tight adhesions are encountered. Gentle dissection is carried out at these tight adhesions to avoid tearing of periosteum. Laterally, the frontozygomatic suture is identified and periosteum elevated to identify and zygomatico-facial and zygomatico- temporal neurovascular bundles which are then cauterized (Figure 8).
Figure 1.
The skin incision for the eyelid sparing orbital exenteration is made 2 mm behind the ciliary margin.
Figure 2.
Two or three traction sutures are placed through the upper and lower tarsi to provide traction on the orbital contents.
Figure 3.
Skin incision is made along the marked area using radiofrequency probe or 15 no blade or cutting diathermy.
Figure 4.
Skin and orbicularis flaps are raised up to bony rim for 3600.
Figure 5.
The periosteum is incised for 360 degrees along the orbital margin.
Figure 6.
The periosteum is elevated from orbital rim up to the apex of the orbit 360°.
Figure 7.
Supra-orbital neurovascular bundle (arrow) is identified and cauterized.
Floor of the orbit is thin and fragile like medial lamina papyracea and dissection has to be gentle so as not to fracture it or create a communication with maxillary sinus. As the lacrimal sac is approached by dissecting medial to it, nasolacrimal duct is divided with diathermy. The exposed end of the nasolacrimal duct is further cauterized with diathermy to obliterate it. This step decreases the risk by of post-operative fistula formation. Next, inferior orbital fissure is encountered and penetrating vessels are divided by cautery. After separation of the periosteum 3600 from the from arcus marginalis to the orbital apex, a pair of curved enucleation scissors are introduced into the posterior orbit. With left hand traction is applied and with the right hand the optic nerve, the superior orbital contents and posterior orbital tissues are divided. Hemostasis is achieved by ice cold wet gauze, pressure and cautery. If necessary additional hemostasis is achieved by surgicel and bone wax. The empty socket is examined meticulously for any residual tumor tissue (Figure 9). Additional apical tissue can be resected if needed and complete hemostasis achieved. Frozen section analysis can be done to assess adequacy of resection. The eyelid flaps are reapproximated with 4–0 vicryl for orbicularis and 6–0 ethilon for skin (Figure 10). Aspiration of the socket for blood or serum is done as and when required. Usually socket heals by 6 weeks and is ready for prosthesis placement by 8 weeks. The main advantage of the lid sparing is early wound healing, better cosmesis, and minimum patient morbidity.
Extended orbital exenteration is a challenge to the surgeon as various modifications need to be made depending on the presenting clinical scenario. All the forms of the extended exenteration are various modifications of the total lid sparing orbital exenteration as described above. We discuss step by step approach in different clinical scenarios depending on the extension of the cancer into various surrounding structures.
7. Orbital exenteration with surrounding skin and soft tissue resection
The demonstrated patient has a pleomorphic rhabdomyosarcoma of the right orbit (Figure 11). Main bulk of the tumor was present in the superior orbit and protruded between the eyelids superiorly (Figure 11). The tumor was infiltrating the soft tissues of the eyelids circumferentially (Figure 11). He had received chemotherapy and radiation as part of initial treatment with disease progression. A preoperative contrast-enhanced CT scan axial and coronal view demonstrates well defined, homogenous and iso-dense soft tissue mass filling the orbit completely (Figure 12). The space occupying lesion was surrounding the globe completely with invasion of the subcutaneous soft tissues overlying the nasal bone and extends up to the lamina papyracea of the right orbit (Figure 12A). There was erosion of the floor of the orbit without extension of the disease into the maxilla (Figure 12B).
Figure 11.
Advanced pleomorphic rhabdomyosarcoma of the right orbit fungating through the palpebral fissure and invasion of the surrounding skin and soft tissues.
Figure 12.
(A) The axial view of contrast enhanced CT scan shows a large soft-tissue homogenous, iso-dense mass involving all the quadrants of the orbit enclosing the globe in middle with eyelid and surrounding soft tissue infiltration. (B) The coronal view of contrast enhanced CT scan shows a large soft-tissue homogenous, iso-dense mass involving all the quadrants of the orbit with eyelid and surrounding soft tissue infiltration.
Assessment of the surrounding skin and soft tissue is done by palpation and lifting of the skin from the underlying structures before marking the incision. Radiology images are carefully reviewed, preferably with radiologist and incision planned accordingly. In this particular case, significant amount of eyelid tissue is infiltrated by the tumor and eyelid preservation is not possible, the incisions here is made full thickness along the orbital rim area at least 1 cm away from the indurated skin margin (Figure 13). Incision is then made along the skin marking, full thickness, as demonstrated, in small increments and complete hemostasis achieved in incised segments to reduce the blood loss and maintain clean surgical field (Figure 14A). This procedure is continued circumferentially 360 degrees along the planned incision (Figure 14B). Once the orbital rim is reached, periosteum is exposed and a fine tip monopolar cutting cautery is used to incise the periosteum circumferentially just outside the orbital rim or arcus marginalis (Figure 15A and B). After incision of periosteum around the rim, Freer periosteal elevator is used to dissect the periosteum off the bony orbit circumferentially (Figure 16A and B). After elevation of the periosteum circumferentially, subperiosteal dissection continued till the orbital apex (Figure 17A and B). Rest of the steps are similar to the total lid sparing orbital exenteration as described above. The lacrimal sac is approached by dissecting medial to it and dividing common canaliculi and orbicularis attachments. It is then dissected from the lacrimal sac fossa and divided from the nasolacrimal duct, preferably with cautery (Figure 18A and B). The exposed nasolacrimal duct is obliterated by cautery to decrease the risk of fistula formation. A pair of curved enucleation scissors are then introduced into the posterior orbit and the optic nerve, superior orbital fissure contents and posterior orbital tissues are cut (Figure 19A). The socket is carefully examined carefully for any residual tumor tissue (Figure 19B). In such a case where generous amount of the eyelids have been sacrificed due to the tumor infiltration, different local or free flaps may be used for reconstruction. Since our case was child of 11 years age, we preferred generous undermining of the surrounding skin flaps (Figure 20A and B) and could approximate primarily, albeit with some tension (Figure 21). Our preference is always to close the orbit with preserved lids or advancement of the cheek skin to decrease the donor site morbidity.
Figure 13.
Eyelid sacrificing skin incision marked for the planned surgical excision in case of involvement of the eyelids.
Figure 14.
(A) The skin incision is full thickness and is deepened in a plane superficial to the periosteum in small increments to achieve complete hemostasis. (B) Skin incision is deepened up to the periosteum circumferentially 3600 around the orbit.
Figure 15.
(A) Periosteum is incised with monopolar cautery along the orbital rim superiorly. (B) Periosteum is incised with monopolar cautery just beyond the orbital rim inferiorly again 360° circumferentially.
Figure 16.
(A and B) Freer periosteal elevator is used to elevate the periosteum of the orbit circumferentially.
Figure 17.
(A and B) Subperiosteal dissection being carried to the orbital apex.
Figure 18.
(A) Lacrimal sac is approached by dissecting medial to it and dividing common canaliculi and orbicularis attachments. (B) Lacrimal sac dissected from the fossa and divided from the nasolacrimal duct.
Figure 19.
(A) The extraocular muscles and the optic nerve are divided at the orbital apex with curved enucleation scissors and specimen delivered. (B) Empty socket after orbital exenteration.
Figure 20.
(A) Generous undermining of the superior skin flap with pericranium kept undisturbed on cranium. (B) Generous undermining of the lateral skin flap.
8. Radical resection for a locally advanced basal cell carcinoma involving floor of the orbit and zygomatic bone
This demonstrated case has long standing basal cell carcinoma of the left lower eyelid with extension into the orbit (Figure 22). The CT scan showed soft tissue mass with enhancement involving left lateral eyelid skin and subcutaneous tissue with extension to the pre-septal and post-septal left orbit. The lesion invades anterior portion of the floor of the orbit and zygomatic bone (Figure 23). The plan of surgical resection includes access through upper eyelid preservation with upper eyelid incision marked 2 mm beyond the eyelash margin, while on the lower lid the involved skin and soft tissue is generously resected en-bloc with the specimen (Figure 24). The upper lid dissection is done between anterior and posterior lamella of the eyelid, while the lower lid incision is full thickness at least 1 cm away from the visible tumor margin. The skin incision is deepened, leaving a generous amount of soft tissue on the lateral and inferior wall of the orbit to secure adequate margins around the tumor. The skin flap is elevated laterally, directly over the zygoma and the anterior bony wall of the maxilla. The upper lid dissection and periosteal elevation is similar to the steps described for above first case. The bone cuts to encompass the tumor are depicted on a skull (Figures 25 and 26). A power saw is used to make bone cuts for the proposed inferior orbital wall and zygoma resection. The inferior rim of the orbit is divided medial to the infraorbital foramen to keep the lower lateral quadrant with the specimen with adequate bone margins (Figure 25). This bone cut extends through the floor of the orbit up to the optic foramen posteriorly. Bone cuts are made on the lateral wall of the orbit at fronto-zygomatic suture and zygomatico-temporal suture (Figure 26). This superior bone cut is extended through to the superior orbital fissure (Figure 25). Small osteotomes are used to complete the bone cuts and to mobilize the bony attachments of the surgical specimen. Once all bone cuts are completed, the surgical specimen remains attached only at the cone of the orbit posteriorly through the attachments of the extraocular muscles and the optic nerve. The orbital contents are retracted laterally and the posterior attachments of the extraocular muscles and the optic nerve are transacted with curved enucleation curved scissors to deliver the specimen (Figures 27 and 28). Rest of the steps are similar to as described for the above case for achieving hemostasis. Specimen and orbital defect is examined after hemostasis is achieved and reconstruction of the defect done.
Figure 22.
Basal cell carcinoma of the left lateral lower eyelid with orbital invasion.
Figure 23.
CT scan showing orbital invasion by periocular basal cell carcinoma. Erosion of the orbital rim and floor is noticed.
Figure 24.
The plan of surgical resection through a upper lid sparing, while generous amount of skin and soft tissues are kept on lower lateral region.
Figure 25.
The inferior rim of the orbit is divided medial to the infraorbital foramen.
Figure 26.
Bone cuts are made on the lateral wall of the orbit at fronto-zygomatic suture (blue circle) and zygomatico-temporal suture (black circle).
Figure 27.
Anterior view of the total orbital exenteration and lateral orbital wall resection specimen.
Figure 28.
Lateral view of the total orbital exenteration and lateral wall removal specimen.
Orbital exenteration with addition of the operative procedure to include part of maxilla is used when the orbital tumors invade adjacent ethmoid sinuses or the nasolacrimal duct system. Various additional surgical procedures are used to remove such tumors depending on the clinical scenario. The demonstrated patient shown in Figure 29, has lympho-epithelial carcinoma of the left lacrimal sac. An axial T1-weighted magnetic resonance imaging (MRI) scan, reveals a multi-lobular, slightly hyperintense lesion in the medial aspect of the right orbit with loss of plane with medial rectus (Figure 30). An axial T2-weighted MRI scan demonstrates space occupying mass in the medial orbit with extension to the ethmoid sinuses (Figure 31). An axial view post-contrast fat-suppressed T1-weighted image shows peripheral enhancement and a lack of central enhancement (Figure 32). The operative procedure therefore will entail an orbital exenteration with a medial maxillectomy. The medial wall of maxilla is accessed through lateral rhinotomy incision that extends from the floor of the nasal cavity along the alar groove and the lateral aspect of the nose up to the medial canthus. Upper and lower eyelid incisions are made 2 mm away from the lid margin and are extended along the nasolabial fold to encompass the involved portion of the skin overlying the lacrimal fossa and nasolacrimal duct (Figure 29). Skin incision and flap elevation up to the periosteum is done as described above in the first case except that a generous portion of soft tissue is sacrificed at the medial aspect of the incision where the skin is involved. Here the skin incision is deepened straight down to the nasal bone medially and the anterior wall of the maxilla laterally. In the inferomedial quadrant of the orbit the soft tissues along the lacrimal sac region are retained on the specimen. No attempt is made to mobilize the periosteum on the lower medial wall of the orbit at the lacrimal apparatus and the lacrimal fossa as medial wall of maxilla will be resected en-bloc with the orbital contents. At the lower end of pyriform aperture of the nose mucosal incision is made with the monopolar cautery till the posterior choana below the inferior turbinate. After periosteal elevation of the superior, lateral and the inferior orbital walls, a oscillating power saw is used to make bone cuts for the proposed medial maxillectomy (Figure 33). The inferior rim of the orbit is divided lateral to the infraorbital foramen to keep the lower medial quadrant in the specimen with adequate bone margins. This bone cut extends through the floor of the orbit up to the inferior orbital fissure and the anterior wall of the maxilla is divided in the plane extending up to the pyriform aperture (Figure 33). Superior bone cut is made above the meridian of the orbit so that entire lacrimal fossa can be resected with the specimen with satisfactory margins. This extends posteriorly along the lamina papyracea up to the posterior ethmoids. Medial bone cut is made on the lateral aspect of the left nasal bone from the orbit up to the nasal vestibule (Figure 33). Inferiorly along the pyriform aperture, osteotome is used to make a bone cut along the lower border of the lateral wall of the nose, below the inferior turbinate. Once all the osteotomies are completed, the surgical specimen remains attached only at the cone of the orbit posteriorly through the attachments of the extraocular muscles and the optic nerve. Rest of the steps are similar as described above. Additional attention is paid to the sphenopalatine artery as brisk hemorrhage may result from it and is easily controlled with electrocoagulation. In this particular case due to adjuvant radiotherapy, necrosis of the skin flaps resulted in the open orbital defect (Figure 34). The surgical defect following healing of the socket shows the medial one-third of the orbital rim has been resected to encompass the tumor of the lacrimal apparatus, which is removed in an en bloc fashion with the contents of the orbit and lateral wall of the nasal cavity (Figure 34). Such cases can be rehabilitated best by prosthetic replacement and glasses as shown in the Figure 35 and Figure 48.
Figure 29.
Clinical picture of lymphoepithelial carcinoma of the left lacrimal sac. Incision encompasses the involved portion of the skin overlying the lacrimal fossa.
Figure 30.
An axial T1-weighted MRI scan, reveals a multi-lobular, slightly hyperintense lesion in the medial aspect of the right orbit with loss of plane with medial rectus.
Figure 31.
An axial T2-weighted MRI scan demonstrates mass lesion in the medial orbit extending to the ethmoid sinuses.
Figure 32.
An axial view postcontrast fat-suppressed T1-weighted image shows peripheral enhancement with lack of central enhancement.
Figure 33.
The planned bone cuts are outlined on a skull superior cut is along the nasal bone and inferior cut is made lateral to the inferior orbital foramen.
Figure 34.
The postoperative appearance of the patient approximately 2 month after surgery shows excellent healing within the orbital socket.
Figure 35.
(A) Rehabilitation of the orbital socket with prosthesis. (B) Glasses are used to enhance appearance.
10. Orbital exenteration with lateral wall removal
This patient has right lateral canthal infiltrative basal cell carcinoma with lateral orbital wall invasion (Figure 36). He had been operated twice before and had also received radiotherapy as part of initial treatment. Coronal view CT scan revealed ill-defined soft tissue mass causing erosion of the lateral wall and infiltration of the orbit and globe (Figure 37A and B). The surgical plan is orbital exenteration with lateral wall removal along with surrounding soft tissues. Upper and lower lid incisions were marked 2 mm beyond eyelid margin with generous amount of skin and soft tissue kept attached to the eyelids at the lateral canthus to ensure satisfactory soft tissue margins (Figure 38). Superior lid, inferior lid and medial dissection is done similarly as described in first case, while laterally generous amount of soft tissues is left for safe margin attached to the specimen. Superiorly, medially and inferiorly subperiosteal dissection is done till the orbital apex as described for above cases. The surgical procedure needs a orbito-zygomatic cranial base exposure. A superior, lateral and inferior orbitectomy is done along with the removal of the zygomatic bone as shown on the skull (Figure 39A and B). Oscillating saw is used to make bone cuts from the superior orbital rim to superior orbital fissure posteriorly. Inferior cut is made from the inferior orbital rim to the inferior orbital fissure posteriorly and zygomatico - temporal suture is divided. Small osteotomes are used to mobilize the surgical specimen after division of the surrounding soft tissues with electro- cautery. Diamond bur can be used to smoothen the bone edges. In a scenario of the erosion of the cranial base in the region, diamond bur can be used to remove the bone without causing tear in the dura. Even appropriate size Kerrison’s punch can be used to remove the cranial base with adequate margins with out injuring dura. The surgical defect after removal of the specimen is examined for completion of surgery (Figure 40) and specimen can be sent for frozen section analysis of soft tissue margins. Anterior and posterior view of the specimen can be seen in the Figure 41A and B. In this particular case since enough of upper and lower eyelid flaps could be preserved, we could achieve primary closure (Figure 42).
Figure 36.
A patient with a basal cell carcinoma of the right lateral canthus with extension into the orbit.
Figure 37.
(A) The coronal view of the CT scan shows ill-defined tumor involving the lateral quadrant of the right orbit with erosion of the lateral wall. (B) 3-D reconstruction of the orbit showing eroded lateral wall.
Figure 38.
Surgical plan of resection with generous amount of soft tissues kept on the lateral wall to achieve disease free margins.
Figure 39.
(A) Anterior view of the planned bone cuts outlined on a skull. (B) Right lateral view of the planned bone cuts outlined on a skull.
Figure 40.
The surgical defect shows the apex of the orbit and remnant walls after resection.
Figure 41.
(A) Anterior view of the surgical specimen shows en-bloc resection of the tumor with the contents of the orbit and the lateral soft tissues. (B) Posterior view of the surgical specimen shows en-bloc resection of the tumor with the contents of the orbit and the lateral soft tissues.
Figure 42.
The skin incision is closed in layers.
11. Orbital exenteration with superior orbital margin/frontal bone removal
The patient presented with adenoid cystic carcinoma of the right lacrimal gland (Figure 43). Contrast enhanced CT scan coronal and axial view reveals soft tissue mass in the upper lateral and superior orbit with outward and medial displacement of the eye ball (Figure 44A and B). Three dimensional reconstruction reveals erosion of the superior orbital rim and outer plate of the frontal bone without any intracranial extension (Figure 45). Such a case in addition to the orbital exenteration will need lateral and superior orbitectomy. Skin incision and periosteum exposure were performed as described for the first case. The periosteum on the superior rim was removed widely to encompass the involved area and achieve negative margins of resection. After orbital exenteration the lateral and the superior orbital wall or cranial base was drilled generously to expose dura completely to ensure even the microscopic tumor is removed. The skull base area to be resected is shown in the Figure 46A and B. The Bone drilling is done well beyond the grossly involved margin and dura exposed (Figure 47). To hasten the process Kerrison’s punch can be used to remove the thinned out bone. Since this is an eyelid sparing procedure, surgical defect is repaired with closure of the remnant eyelid flaps.
Figure 43.
This patient has an adenoid-cystic carcinoma of the right lacrimal gland.
Figure 44.
(A) A contrast-enhanced coronal view of CT scan shows the tumor involving the superior and lateral orbit with displacement of the globe. (B) A contrast-enhanced axial view of CT scan shows the tumor involving the orbit.
Figure 45.
3-D reconstruction revealed erosion of the orbital rim and outer plate of the frontal bone.
Figure 46.
(A) Anterior view of the planned bone cuts outlined on a skull. (B) Right lateral view of the planned bone cuts outlined on a skull.
Figure 47.
The surgical defect shows the complete removal of the superior and lateral wall of the orbit and exposed dura (arrow).
12. Orbital exenteration with total maxillectomy
This patient has squamous cell carcinoma of the right maxillary sinus invading the orbit through the periosteum (Figure 48). Contrast enhanced CT scan of the paranasal sinuses reveals soft tissue mass involving the right maxillary sinus completely and eroding floor of the orbit and extending into the orbit through the periosteum (Figure 49). To remove the tumor en-bloc, orbital exenteration with total maxillectomy is indicated. Orbital exenteration of a functioning eye with normal vision is only indicated if the procedure is done with curative intention. A lateral rhinotomy incision with midline lip is split is extended laterally as upper and lower lid incisions circumferentially encompassing the palpebral fissure of the eye (Figure 48). The skin incision begins in the midline of the upper lip up to the root of the columella. Here the incision extends into the floor of the nasal cavity and then returns back outside of the nasal cavity around the ala of the nose up to the medial canthus to join circumferential orbital incision (Figure 48). The skin incision is deepened through the subcutaneous tissues and musculature of the upper lip and the right cheek. The cheek flap is elevated laterally with a mucosal incision along the upper gingivobuccal sulcus. The skin incision for the sub-ciliary extension begins at about the level of the medial canthus of the eye 2 mm beyond the eyelid margin. The skin incision here should be placed meticulously without tearing as the skin over the eyelid is thin. The cheek flap is elevated to about 1 cm beyond the lateral canthus to provide adequate access to the anterolateral wall of the maxilla. After elevation of the cheek flap, attachment of the orbital periosteum to the orbital rim is incised in its superior half. A periosteal elevator is used to separate the orbital periosteum from the bony roof of the orbit all the way up to the apex of the orbit. Periosteum of the lower half of the orbit is kept intact, so as not to violate the surgical field. The attachment of the masseter muscle on the inferior border of the zygoma is divided next with use of the cautery.
A mouth gag is placed on the contralateral side to open the oral cavity and a tongue depressor is used to depress the tongue. A mucosal incision is made between the lateral incisor and the canine tooth, which marks the anterior line of resection of the alveolar process of the maxilla. An incision is now made in the mucosa of the hard palate along midline from the junction of the soft and hard palate and it is further extended to the incision of the alveolar process between the canine and lateral incisor (Figure 51). Posterior end of the midline palatal incision is turned laterally behind the maxillary tubercle to connect the upper gingivobuccal-sulcus incision. This incision is deepened through the mucoperiosteum of the hard palate. Posteriorly the incision is deepened through the attachments of the medial pterygoid muscle to free up soft-tissue attachments to the maxilla.
Nasal vestibule is opened through the piriform aperture to expose the nasal process of the maxilla. All the soft tissue attachment of the maxilla and the orbit are thoroughly divided before proceeding for bone cuts. All the bone cuts are marked by electrocautery. Superior bone cut is through the nasal process of maxilla, laterally the maxilla is separated from the zygomatic arch along the inferior orbital fissure and inferiorly the maxilla is divided through its alveolar process between the lateral incisor and canine tooth up to the midline to the posterior margin and from there onward through the midline up to its posterior margin (Figures 50 and 51). Posterolateral wall is separated from the pterygoid plates through its hamulus by placing a curved osteotome in between and gentle tap with mallet (Figure 52). All the bone cuts are accomplished by oscillating power saw. Once all the bone cuts are completed with the power saw, an osteotome is used to mobilize the specimen en-bloc (Figures 53–55). Soft-tissue and muscular attachments on the posterior aspect of the maxilla are divided with heavy curved scissors. The surgical defect following total maxillectomy with orbital exenteration is shown is shown in (Figures 55 and 56). Surgical defect was reconstructed by primary closure and prosthetic rehabilitation (Figure 57).
Figure 48.
Clinical picture of advanced squamous cell carcinoma of the right maxillary sinus invading right orbit.
Figure 49.
Coronal CT scans show a large left maxillary sinus mass that has destroyed most of the sinus walls. The tumor extends into the orbit through the periosteum.
Figure 50.
Medial and lateral bone cuts shown on skull.
Figure 51.
Palatal bone cuts shown on skull.
Figure 52.
Posterior separation line between maxilla and pterygoid plates.
Figure 53.
A anterolateral view of the specimen.
Figure 54.
A lateral view of the specimen.
Figure 55.
A palatal view of the specimen.
Figure 56.
Surgical defect with cheek flap.
Figure 57.
Surgical defect with retracted cheek flap.
13. Reconstruction following extended orbital exenteration
Reconstruction of the defects that result from extended orbital exenteration is a challenge. There are basically two methods, first is open method and second is closed method (Table 4). In open method, healing occurs by secondary intention, as the depth of the orbit is left open for granulation with regular dressings [18]. This is time consuming process and this option is suboptimal if the patient is to have postoperative radiation which in most cases needs to be started within 4 weeks post-surgery [18]. Other option is to layer the orbital cavity with a split thickness skin graft. It is usually harvested from the thigh with a humby’s knife or a dermatome, placed directly on the bone and sutured to the skin edges around the socket. It maintains a deeper socket in comparison to the secondary healing as it reduces wound contracture. Due to donor site morbidity and failure of graft uptake in particularly diabetic patients, split thickness grafts are used in conjunction with other flaps like pericranial periosteal flaps from forehead. Since pericranial are vascular, it enhances the uptake of split thickness skin graft.
In Closed method of reconstruction, the orbital cavity is reconstructed either with the residual preserved lid, cheek advancement, locoregional flaps or microvascular free tissue transfer [19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30]. We prefer to close orbit directly in case of adequate amount of lid tissue has been preserved or with cheek advancement. It is the modification of the cervicofacial flap and offers a one-stage, reliable, and safe method of reconstruction following orbital exenteration [19]. Subcutaneous cheek dissection can be performed to various levels usually to a level just below the oral commissure and 2–3 cm below the angle of the mandible avoiding injury to the branches of facial nerve. It can also be used in conjunction with other methods of reconstruction like peri-cranial flap. Other loco-regional options include: a cheek fascio-cutaneous V-Y flap, galeal flap, pericranial flap, cutaneous flap from the forehead and temporalis muscle flap [31, 32, 33]. Cutaneous forehead flap based on the frontal branch of the temporal artery described by Rodrigues ML et al. can be used effective to cover the orbital defect [20]. Apart from being a single stage procedure, this flap can obliterate the orbital defect immediately and adequately [20]. This method is easy, less time consuming and flap has a reliable blood supply and is reproducible. In cases where lateral orbital wall has also been resected, temporalis muscle flap can be used to reconstruct the orbital defect [21]. Menon NG et al. illustrated the method where temporalis muscle can be used to reconstruct the orbital defect with intact lateral wall of the orbit [30]. They transposed entire temporalis muscle to orbit after creating a large window in the lateral orbit, without resection of the lateral orbital rim [30]. Prefabricated myocutaneous - temporalis muscle flap was describe by Altindas M et al. for the reconstruction of eyelids and periorbital skin [22]. Scalp skin island is kept attached to the temporalis muscle for the reconstruction of lid margins and eyelashes and the neighboring bare temporoparietal fascia is used for the augmentation of the periorbital soft tissues [22]. Microvascular free tissue transfer is the ideal reconstructive option for the large and complex defects particularly resulting from extended orbital exenterations. Various free flaps like anterolateral thigh, radial fore-arm, parascapular, rectus abdominis muscle and gracilis muscle free flaps have been described for orbital reconstruction [25, 26, 27, 28, 29]. The modified radial forearm flap described by Purnell et al. provides abundant thin, pliable tissue, facilitating resurfacing of the entire orbit [29].
All the reconstructive methods described till now provide a good cover only and prosthetic reconstruction is needed to improve the appearance beyond that of an eyepatch. Using only prothesis without osseointegration have drawback like poor fit and discoloration over time [33, 34]. With introduction of the osseo-integrated implants, there has been a significant improvement in fixation of prosthetics, with associated improvements in quality of life and compliance [35, 36, 37, 38, 39, 40].
14. Conclusions
Extended orbital exenteration although highly disfiguring procedure give excellent chance of controlling the aggressive malignant process. A single procedure does not fit all the clinical scenarios. We have to examine the tumor extension and plan the procedure accordingly. There are many other clinical presentations like intracranial extension etc. We have purposefully restricted our discussion to only cases where dura was not infiltrated, for the readers to concentrate on the scenarios described above. Our efforts will continue to publish more such research to benefit our readers in understanding the surgical management of orbital tumors. Reconstruction of complex defects following extended orbital exenteration remain a challenge. Locoregional cutaneous and muscle flaps are useful, but free flaps like radial forearm flaps not only prove a good reconstruction option but tolerate the adjuvant treatment like radiation well. Last but not least, prosthesis helps in further enhancing the appearance and rehabilitation.
Acknowledgments
We are thankful to all the patients who have put trust in our abilities to treat them at our Apollo cancer center, Hyderabad, India. We are also grateful to Dr. Vijay Anand Reddy, Director Apollo cancer center, for constant encouragement to our scientific endeavors. This chapter would not have been possible without his valuable scientific inputs.
Conflict of interest
The authors declare no conflict of interest.
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Written By
Arsheed Hussain Hakeem, Hassaan Javaid, Novfa Iftikhar and Usaamah Javaid
Submitted: 24 September 2021Reviewed: 31 March 2022Published: 16 September 2022
Open access peer-reviewed chapter
