**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**).

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

#### **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.*

#### **Figure 13.**

*Eyelid sacrificing skin incision marked for the planned surgical excision in case of involvement of the eyelids.*

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

#### **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.*

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 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.*

**Figure 21.** *Primary closure of the surgical defect.*
