Ординатура / Офтальмология / Английские материалы / Surgical Atlas of Orbital Diseases_Mallajosyula_2009

CASE ILLUSTRATIONS

Case 1

Female 48 years (Figure 21.24A) presented with eccentric proptosis of 2 years duration, and defective vision since 2 months. She gives history of recurrent episodes of sinusitis. On examination, she had eccentric proptosis of left eye with the globe pushed up (6 mm), outwards (7 mm) and forwards by 7 mm.The ocular motility was restricted mildly in all directions. Pupil showed RAPD, and her BCVA was 20/60 and color vision was 6/17. Fullness of left cheek was noted. CT scan of orbit (Figures 21.24B

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and C) showed a large, hyperdense lesion filling entire maxillary sinus, with orbital extension. Extension in front of maxilla into cheek was found. The optic nerve was pushed by the mass. In view of recurrent sinusitis, involvement of the sinuses, fungal granuloma was the clinical diagnosis. The lesion was debulked through a modified lateral rhinotomy incision, along with my ENT colleague. The orbital component was debulked through the horizontal incision, exposing the floor of the orbit. She received amphotericin-B for the fungal granuloma. Her vision improved to 20/30, and there was no recurrence in the past 4 years.

Case 2

Female of 16 years presented with eccentric proptosis of left eye since 1 year and blurring of vision since 1 month. She was an agricultural worker. She had an eccentric proptosis of left eye with the eyeball pushed up and out (Figure 21.26A). Ocular motility was restricted in elevation and abduction pupil showed RAPD in the left eye. Her BCVA was 20/40. CT scan (Figures 21.26B and C) showed a mass lesion of the

maxillary and ethmoid sinuses, with bony destruction and orbital extension. Since most of the orbital floor was destroyed, we planned for a swinging lower eyelid approach, which gives adequate exposure to the floor of orbit (Figure 21.27A) and the mass was debulked (B). Postoperative recovery was good (C). However there was significant enophthalmos. She improved with antifungal drugs and there was no recurrence during 3 years follow-up.

Case 3

A 22 years male presented with complaints of left sided nasal obstruction since 5 months, bleeding from the left nasal cavity since 4 months, swelling over the left cheek since 3 months and protrusion of the left eye since 3 months. On anterior rhinoscopic examination a pinkish mass was seen in the left nasal cavity extending into the posterior choana which was observed in the posterior rhinoscopy. There was a

proptosis of the left eye (Figure 21.28A). Ocular movements and vision were normal. CT scan showed a space occupying lesion arising in the left maxillary sinus which is extending into the nasal fossa causing displacement of inferior and medial walls of the orbit s/o neoplasm (Figure 21.28B). The mass was excised through the Moure's lateral rhinotomy approach. Histopathology showed it to be nasopharyngeal angiofibroma (Figure 21.28C and D).

Figures 21.28A and B: Eccentric proptosis of left eye with the globe pushed up. Note the fullness below the lower eyelid (A) CT scan (B) shows a hyperdense mass with contrast enhancement, involving the maxillary sinus, extending into the nasal cavity. Note the bony expansion of the sinus, leading to proptosis

Case 4

Boy of 2 years was brought with complaints of right sided nasal obstruction since 2 months, protrusion of the right eye since 1 month and watering of the right eye since 1 month (Figure 21.29A). On anterior rhinoscopic examination a grayish mass was seen in the right nasal cavity. Right eye was proptosed. Ocular movements were normal. CT scan (Figure 21.29B) revealed a well defined, non enhancing lesion in the right anterior ethmoidal air cells with central hyperdense component causing expansion of adjacent

Figures 21.29A and B: Eccentric proptosis of right eye (A) with the globe pushed laterally and down. Note the fullness of superior sulcus.

CT scan (B) shows a large, heterogenous lesion involving the ethmoid sinuses, with bony expansion

Figures 21.28C and D: Histopathology showing a picture of vascular spaces with less stroma of fibrous tissue (C) suggestive of Angiofibroma (D) shows postoperative picture with recovery from proptosis

bony structures and proptosis of the right eye. The mass was excised through medial orbitotomy approach and the mass was sent for HPE which showed infected granuloma (Figures 21.30A and B).

Case 5

Male 15 years, presented with complaints of left sided nasal obstruction since 5 months and protrusion of the left eye since 3 months (Figure 21.31A). On anterior rhinoscopic examination a grayish white mass was seen in the left nasal cavity. There was eccentric proptosis of the left eye. Ocular movements and visual acuity were normal. CT scan showed a large well defined soft tissue lesion seen in the region of the ethmoidal air cells on the left side extending into left nasal cavity and sphenoid sinus. The lesion was surrounded by thick irregular sclerotic bony margins and there was expansion of the bony outlines into the left orbit causing proptosis, suggestive of fibrous dysplasia. In view of his symptoms and cosmetic considerations, the mass was excised through external ethmoidectomy approach. Histopathology confirmed the diagnosis of fibrous dysplasia (Figures 21.32A and B).

Figures 21.30A and B: Excised mass (A) and the early postoperative recovery (B)

Figures 21.31A and B: Note the eccentric proptosis with the globe pushed outwards (A) CT scan revealed an expansile lesion of the ethmoid bone (B) with sclerotic margins, heterogenisity and cystic spaces within, suggestive of fibrous dysplasia

Figures 21.32A and B: Microphotography showing well demarcated islands of cartilage with interwoven fibro-osseous tissue, suggestive of fibrous dysplasia (A) The early postoperative recovery with improvement in proptosis is evident (B)

Case 6

Female 34 years, presented with proptosis of right eye of 6 months duration, and defective vision since 1 month. She had axial proptosis of 5 mm and

A

downward displacement by 4 mm (Figure 21.33A). RAPD was observed. Her BCVA was 20/30 in right eye. The past history was significant in that she had convulsions 2 years back, and was using anticonvulsants since then CT scan of the orbit revealed an intraconal, heterogenous, apical mass with very distinct margins (Figures 21.33B and C). The CT scan of brain (Figures 21.34A to C) showed calcified lesions without any surrounding edema in the frontal and occipital cortex. The possibility of hemangioblastoma was considered. Fundus examination was normal. In view of the apical location, a transcranial approach (A) was performed (Figures 21.35A and B). Histopathology confirmed it to be hemangioblastoma. There was no recurrence in the 2 years follow-up.

Figures 21.35A and B: In view of the apical location, a transcranial approach (A) was performed. Histopathology confirmed it to be hemangioblastoma. Postoperative figure (B) showing complete recovery

BIBLIOGRAPHY

1.Atallah N (1981) osteomas of the paranasal sinuses, journal of laryngology and otology.

2.Balasingam V, Noguchi A, McMenomey SO, Delashaw JB Jr Modified osteoplastic orbitozygomatic craniotomy. Technical note J Neurosurg. 2005;102(5):940-4.

3.Bordley, JE nad Bosley, mucoceles of the frontal sinus, annals of otology, rhinology and laryngology.

4.Canalis RL, ethmoidal mucocele. Archives of otolaryngology.

5.Cheesman AD Lund, Cranio facial resection for tumors of nasal cavity and paranasal sinuses.

6.Doxanas MT Clinical Orbital Anatomy.

7.Eichel the intranasal ethmoidectomy procedures.

8.Fearon BEdwards orbitalfacial complications of sinusitis in children.

9.Freedman HM (1979) complications of intranasal ethmoidectomy.

10.Fu JD, Zhao JW, Yin DL, Liu HC, Qiu E, Zhang JL, Zhang TM. Surgical treatment of fibrous dysplasia of the skull with neuro-navagation .Zhonghua Yi Xue Za Zhi. 2004;17;84(10):808-12. Chinese.

11.Goisis M, Biglioli F, Guareschi M, Frigerio A, Mortini P Fibrous dysplasia of the orbital region: current clinical perspectives in ophthalmology and cranio-maxillofacial surgery. Ophthal Plast Reconstr Surg. 2006;22(5):383-7.

12.Harrison DFN (1971) surgical anatomy of maxillary and ethmoid sinuses.

13.Harrison DFN (1980) The ENT surgeon looks at the orbit, journal of laryngology and otology.

14.Harrison DFN (1981) Surgical approach to the medial orbital wall. Annals of otology,rhinology and laryngology.

15.Harrison DFN (1987) juvenile angiofibroma, archives of otolaryngology-head and neck surgery.

16.Hejazi N, Hassler W, Offner F, Schuster A. Cavernous malformations of the orbit: a distinct entity? A review of own experiences. Neurosurg Rev. 2007;30(1):50-4; discussion 54-5. Epub 2006.

17.Howard and Lund, the midfacial degloving approach to sinonasal disease, journal of laryngology and otology.

18.Howard, DJ Lund reflections on the management of adenoid cystic carcinoma of nasal cavity and paranasal sinuses. Otolaryngology 93.

19.Hubert (1937) orbital infections due to nasal sinusitis, New York journal of medicine37.

20.Korinth MC, Ince A, Banghard W, Gilsbach JM. Follow-up of extended pterional orbital decompression in severe Graves’ ophthalmopathy. Acta Neurochir (Wien). 2002;144(2):113-20; discussion 120.

21.Linnet J, Hegedus L, Bjerre PK. Neurosurgical treatment of patients with severe thyroid-associated ophthalmopathy. Transcranial two-wall orbital decompression Ugeskr Laeger. 2002;6;164(19):2505-8. Danis

22.Lloyd(1988) Diagnostic imaging of nose and paranasal sinuses.

23.Lund, tomors of paranasal cavities, Oto-Rhino-Laryngology 45.

24.Natvig K and Larsen, mucoceles of paranasal sinuses, Journal of Laryngolog and Otology.

25.Reisch R, Perneczky A. Ten-year experience with the supraorbital subfrontal approach through an eyebrow skin incision. Neurosurgery. 2005;57(4 Suppl):242-55; discussion 242-55.

26.Rontal (1979) Surgical anatomy of the orbit, annals of Otology, Rhinology and Laryngology 88.

27.Schramm, Orbital complications of sinusitis, Otolaryngology, 86.

28.Zizmor(1968) cysts and benign tumors of the paranasal sinuses,, seminars in Roentgenology.3

Exenteration refers to removal of the eyeball along with the orbital contents. This disfiguring and destructive procedure is reserved for the treatment of life-threatening conditions where other approaches have failed.

The first mention of orbital exenteration was 400 years ago by Bartische in his treatise on eye diseases.1 He described partial exenteration. Extensive orbital exenteration was described by Golovine, Nowikoff and Filatov in the early 1900s.2

Indications

We usually perform this procedure in the following situations.

1.Malignancies

a.Primary orbital lesions like extensive adenoid cystic carcinoma of the lacrimal gland

b.Intraocular lesions like orbital extension of retinoblastoma, choroidal melanoma

c.Orbital extension of periocular malignancies

i.Carcinoma of the paranasal sinuses

ii.Eyelid tumors

iii.Skin malignancies

2.Infections like sino-orbital mucormycosis and other fungal infections.

3.Relative indications

a.Severe orbital contracture with inability to wear a prosthesis

b.Neurofibromatosis with orbital deformity

c.Orbital meningioma and lymphangioma causing disfiguring proptosis

d.Recalcitrant orbital inflammations unresponsive to other treatment modalities

Patient Preparation

The patient needs to understand the need for such a destructive surgery and the gross disfigurement caused by the procedure. We also counsel our patients extensively that their facial appearance can never be restored to what it was before. However, an orbital prosthesis can restore appearance to an acceptable extent, although it will never provide eyelid or ocular motility. Psychological help is sometimes sought when we feel that the patient may not be able to withstand the consequences of the surgery.

Surgical Procedure

We always perform the procedure under general anesthesia. In the rare situation when this is not possible, local anesthesia can also be used with 2% lignocaine with 1:200,000 adrenaline given as a retrobulbar injection and followed by injections around the orbital rim and in addition with infraorbital, nasociliary and frontal nerve blocks. For every patient we have a pint of blood ready for transfusion if required. In addition hypotensive anesthesia is used for all the cases. We also ensure that the cauteries are working well and have sufficient gauze on table to control bleeding.

The skin is marked all around with a methylene blue marker. A 4-0 silk suture is passed through the eyelid skin and orbicularis to exit from the tarsal

plate at the lid margin and then passed similarly through the tarsal plate and skin and tied to secure the lids together. This suture is left long to provide traction during the procedure.

A lid sparing exenteration is less disfiguring than a total exenteration. In such a case we make the incision a short distance away from the lid margin. If however there is involvement of the lid, the incision is made just inside the orbital rim. The skin incision is made with a 11 no. Bard Parker knife, starting inferiorly and progressing laterally, superiorly and medially. The superonasal quadrant is approached last as it bleeds extensively. If the lid skin is being spared, it is dissected along with orbicularis to reach the periosteum of the orbital rim. Once the lid skin is cut, the underlying tissues can then be cut with a monopolar cautery.

The periosteum is cut 6 millimetres or so from the orbital rim and then elevated using a periosteal elevator. The sharp end of the periosteal elevator is used to separate it from the orbital rim and the blunt end for the intraorbital portion. The attachments in the region of the medial and lateral canthal tendon and the trochlea are lifted off from the underlying bone. The neurovascular bundle entering through the infraorbital and supraorbital foramen is usually cauterised before cutting it. We separate the

periorbita from the bone superiorly, laterally and inferiorly first using the blunt end of the periosteal elevator or a lens spatula. The periorbita is firmly attached to the bone near the orbital rim and loosely within the bony orbit. Medially the lacrimal sac is elevated and cut near the nasolacrimal duct. Occasionally if there is a suspicion of tumor spread through the nasolacrimal duct we remove bone in that region using the Kerrison's rongeur. In cases of tumors where there is bone involvement, we remove all the involved bone. If such a step is pre-empted, then occasional help from a neurosurgeon for tumors extending superiorly and sometimes our ENT colleague especially for sino-orbital mucormycosis is sought.

Once we reach the orbital apex, we are wary of the vessels that might bleed before we finally remove all the contents of the orbit. We clamp the tissues near the apex with a curved artery forceps before cutting the apical stump. For visualization, we use a lid spatula to push the contents to one side. Bleeding can be a problem and we use a strong cautery to stem it. Bone wax is needed for the occasional recalcitrant bleeder. We wait for the blood pressure to return to normal before we close the lid skin (if performing a lid sparing exenteration). We use absorbable 6-0 vicryl for the underlying tissues and

320 Surgical Atlas of Orbital Diseases

6-0 prolene continuous suture for the skin. If the lid skin has been removed, the socket is packed with Betadine soaked gauze.

We give the patient systemic antibiotics and antiinflammatory medication and give a pressure patch for 2 days. On the third day following surgery we remove the patch and perform daily aspiration of the contents ( in lid sparing) or cleaning and packing in total exenteration.

Management of the Exenterated Socket

It is important to note that the primary objective of exenteration is to remove all disease. Even after exenteration, the patient needs to be monitored carefully clinically or by imaging for any recurrence.

Spontaneous Granulation

This has to be performed when the surgical excision has been radical and the lid skin excised. In these cases we leave the remaining skin to line the socket and pack the socket with gauze soaked in Betadine as a pressure pack and for about 2 weeks with regular changing of dressings in the hospital, or by the patient at home. The entire socket is allowed to heal by granulation. Of course the advantage of this is the fact that any recurrent disease can be identified easily. However the healing is a slow process.

Skin Grafting

Partial thickness skin grafts have been in vogue for a long time since they were popularized by Wheeler.4 Using a dermatome, skin is harvested from a non hair bearing area such as the inner thigh and abdomen. The graft is then fitted to the exenteration cavity, trimmed and sutured using 6-0 prolene sutures. Slits are made to facilitate drainage and prevent collection of fluid underneath. Healing usually takes 6 weeks, following which the patient is fitted with prosthesis.5

Skin Flaps

This is my preferred technique where the lid skin is spared and used to close the cavity. Postsurgery pressure patch is placed and aspiration of the socket is performed daily for a week to remove any collection of fluid. Once the healing is complete, a smooth surface is achieved over which prosthesis can be placed easily.

Myocutaneous Flaps

Other techniques like temporalis muscle transposition, forehead, cheek, pectoralis flaps have also been described.6-9

Prosthesis

Exenteration prosthesis is our preferred technique of managing the cosmetic issues following exenteration, which is discussed as a separate chapter in the book.

Complications of Exenteration

Bleeding can be life threatening especially during the surgery and adequate support from the anesthetic team is a must. In addition blood for transfusion should be available. One should have a good cautery machine.

Socket infection can occur and needs to be managed by systemic and local antibiotics. Any gape in the sutured wound should be closed if possible or in the presence of infection should be allowed to granulate spontaneously.

Recurrence of the primary disease can occur and hence monitoring at regular intervals postsurgery is essential.

CASE ILLUSTRATIONS

Case 1

A 51-year-old female presented with pain and swelling of the left eye for 15 days. She had a history of gradual loss of vision in the left eye for 2 years followed by gradual protrusion of that eye for 1 year. On examination, there was proptosis of the left eye, with swelling of the lids and ptosis (Figure 22.1A). On lifting the ptotic lid, a brown, firm perilimbal nodule was seen next to an opaque cornea (Figure 22.1B). There was complete restriction of eye movements. CT scan of the orbit showed a soft tissue mass filling the orbit almost upto the apex (Figures 22.1C and D). The globe contour was distorted and no normal orbital structures were recognizable. A diagnosis of uveal melanoma with secondary orbital extension was made. There was no lymphadenopathy in the head and neck region. Systemic workup was done which did not reveal any systemic metastasis.

The patient underwent a lid-sparing exenteration. The floor and medial wall bones were deficient because of pressure effect, but the periorbita was

Steps of Surgery (Figures 22.1E to Q)

Orbital Exenteration 321

intact. Subsequently, the patient underwent external beam radiotherapy to the left orbit and paranasal sinuses.

Figure 22.1A: A 51-year-old lady presented with proptosis of the left eye with associated lid swelling and ptosis

Figure 22.1B: A black mass was seen to be prolapsing out of the cornea of the left eye suggestive of extraocular spread of melanoma

Figure 22.1E: The skin was marked on both the upper and lower lids to identify the site of incision

Figure 22.1G: 3 such sutures are passed and the ends kept long for traction during the procedure

Figure 22.1F: A rolled up wet cotton gauze was placed over the conjunctiva. A 4-0 silk suture is passed through the eyelid skin and orbicularis to exit from the tarsal plate at the lid margin and then passed similarly through the tarsal plate and skin and tied to secure the lids together

Figure 22.1H: The skin incision is made with a 11 no. Bard Parker knife, starting inferiorly and progressing laterally, superiorly and medially

Figure 22.1I: In a lid sparing exenteration, the skin incision is made a short distance away from the lid margin

Figure 22.1J: The underlying tissues are retracted and cut with a radiofrequency monopolar cautery