Ординатура / Офтальмология / Английские материалы / Oculoplasty and Reconstructive Surgery Made Easy_Garg,Touky, Nasralla_2009
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SGC and Muir–Torre’s Syndrome 499
Figures 2A and B
ROLE OF FNAC
The neoplasm is known to masquerade as other benign and less malignant lesions, resulting in delay in diagnosis and relative high morbidity and mortality. Fine needle aspiration cytology (FNAC) of recurrent upper eyelid nodules treated elsewhere as chalazion is advocated by the chief author. Cytological smears suggestive of malignancy are subsequently subjected to histopathology and the role of FNAC in early diagnosis and subsequent appropriate surgical management of eyelid sebaceous gland carcinoma to prevent recurrence and metastasis cannot be underscored.
Sentinal Node Biopsy20
sentinel lymph node (SLN) are identified by using technetium Tc 99m sulfur colloid as a tracer. 33% have regional lymph node metastasis. The false-negative rate is higher than that reported for SLN biopsy at most other anatomic sites. Patients with negative findings from SLN biopsy still require careful long-term follow-up because they may develop regional or distant metastasis.
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TREATMENT
Treatment According to Type23
Group A—Sebaceous gland carcinoma with conjunctival intraepithelial (pagetoid) invasion,
Orbital exenteration may be necessary in 36% in group A.
Group B—Sebaceous gland carcinoma without conjunctival intraepithelial invasion.
Historic data indicate a nearly 30% local recurrence rate with standard surgical excision. Excision by means of Mohs micrographic surgery is more efficacious.
ADJUVANTS
Topical Mitomycin24
Topical 0.04% mitomycin-C four times daily is administered for 1 week followed by 1 week off medication. The treatment cycles is repeated until resolution of the conjunctival malignancy is clinically evident.
Neoadjuvant Treatment 26,27
Neoadjuvant chemotherapy, using a combination of carboplatin and 5-fluorouracil.
Eyelid-sparing orbital exenteration may be performed after 3 cycles of chemotherapy, followed by radiotherapy to the regional lymph nodes.
Radiotherapy31-33
> 55 Gy.
a.Tumor too large for resection.
b.Palliative and for secondaries.
But it is important to keep in mind that radiotherapy may induce malignancy in benign tumors.28,31
SGC and Muir–Torre’s Syndrome 501
Reconstruction
A.Cutler-Beard bridge flap—Full-thickness eyelid defects are conventionally reconstructed by either a Hughes flap or a Cutler-Beard bridge flap.
B.The Cutler-Beard bridge flap technique with use of donor sclera for upper eyelid reconstruction
C.The switch flap is an alternative method and is not very widely practiced. The technique involves switching a fullthickness flap on a pedicle to fill a defect from lower lid to upper or vice versa. The pedicle is divided in three weeks. The recipient lid is reconstructed by direct closure and the donor lid by direct closure with or without cantholysis or sliding flap, or it is left to heal by second intention.
D.Double-bridged flap reconstruction of the upper eyelid that spares the eyelid margin can provide excellent functional and cosmetic results, particularly in cases of nonmarginal eyelid tumor excision
E.Orbicularis oculi myocutaneous advancement flap for upper eyelid reconstruction—Two-stage reconstruction of partial or total full-thickness upper eyelid defects. In the first stage, a single tarsoconjunctival flap from the donor lower eyelid reconstitutes the posterior lamella, and a fullthickness skin graft reconstructs the anterior lamella. The tarsoconjunctival flap is incised 1.5 to 2 mm from the lower eyelid margin rather than the 4 to 6 mm necessary to preserve the marginal artery in the Cutler-Beard procedure. In the second stage, 5 to 8 weeks later, the skin tarsoconjunctival flap is severed
F.Hard palate mucoperiosteal graft for posterior lamellar reconstruction of the upper eyelid
G.Moreker’s surgery of direct opposite lid split with lid sharing with semicircular flap (Figures 3A and B).
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Figures 3A and B
In the Cutler-Beard procedure, the full-thickness lower lid is sutured by advancement into the upper eyelid defect. In our procedure which is different from this the upper lid is excised with wide margin with intraoperative frozen section support and then reconstructed with a semicircular flap opposite to the Tenzel’s flap moved at the lateral canthus as seen in the photographs. The remaining gap is filled by a lid split of the lower lid and suturing of lower lid tarsus to the levator and the lower lid anterior lamella being sutured anteriorly to the residual skin orbicularis complex superiorly to keep the eyes closed for a period of 6 weeks. At the end of 6 weeks the lid is split to give excellent lid closure and upper lid function with good cosmetic results.
The evaluation of good reconstruction includes contour of lid, condition of wound, cornea and donor site were examined for infection, dehiscence, corneal ulcer, notching, trichiasis, and recurrence. The preoperative and postoperative photographs are also compared
MORTALITY
Tumor-related deaths occur in only in 6.7%, which is lower than previous reports and may be related to earlier detection or improved surgical excision.
SGC and Muir–Torre’s Syndrome 503
Metastasis
•Risk of tumor-related metastases is similar in both groups
•Lung metastasis is known from meibomian gland carcinoma of eyelid.
•Metastatic adenocarcinoma to the retina has been reported in a patient with Muir-Torre syndrome.
•Radical neck dissection may be needed if neck nodes involved.
FACTORS ASSOCIATED WITH POOR PROGNOSIS34
1.Vascular, lymphatic, orbital invasion.
2.Both lid involvement and multi-centric origin.
3.Poor differentiation.
4.Duration of symptoms more than 6 months.
5.Tumor diameter more than 10 mm.
6.Highly infiltrative pattern and pagetoid invasion of epithelium.
Screening for MTS
The immunohistochemical testing for MSH-2, MSH-6, and MLH-1 is useful for rapid identification of an underlying mismatch repair defect and early diagnosis of MTS.9
59% of sebaceous neoplasia exhibit a mutation in at least one mismatch repair protein gene. The positive predictive value of each is as follows: MLH-1 88%, MSH-6 67% and MSH- 2 55%.
FOLLOW UP GUIDELINES13
Evidence indicates that for individuals with or at risk of MTS or HNPCC, colonoscopy every 1-2 years beginning at age 2025 or 10 years younger than the youngest age at diagnosis in
504 Oculoplasty and Reconstructive Surgery
the family can be strongly recommended. Additionally, most experts believe that an annual history and physical examination, including a complete skin examination and urinalysis, as well as periodic endometrial sampling and/or transvaginal ultrasound for women, are worthwhile screening tests for these high-risk patients.
RECENT ADVANCES
Switching from tacrolimus to sirolimus halts the appearance of new sebaceous neoplasms in Muir-Torre syndrome.
REFERENCES
1.Ramirez CC, Berman B. Cutaneous signs and syndromes associated with internal malignancies. Skinmed. 2005;4:84-90; quiz 91-82.
2.Dores GM, Curtis RE, Toro JR, Devesa SS, Fraumeni JF Jr. Incidence of cutaneous sebaceous carcinoma and risk of associated neoplasms :insight into Muir-Torre syndrome. Cancer. 2008 Oct 17.
3.Barana D, van van, Wijnen J, et al. Spectrum of genetic alterations in Muir-Torre syndrome is the same as in HNPCC. Am J Med Genet A. 2004;125:318-9.
4.Ponti G, Losi L, Di Gregorio C, et al. Identification of Muir-Torre syndrome among patients with sebaceous tumors and keratoacanthomas: role of clinical features, microsatellite instability, and immunohistochemistry. Cancer. 2005;103:1018-25.
5.Popnikolov NK, Gatalica Z, Colome-Grimmer MI, et al. Loss of mismatch repair proteins in sebaceous gland tumors. J Cutan Pathol. 2003;30:178-84.
6.Goldberg M, Rummelt C, Foja S, Holbach LM, Ballhausen WG. Different genetic pathways in the development of periocular sebaceous glandcarcinomas in presumptive Muir-Torre syndrome patients.Hum Mutat. 2006 Feb;27(2):155-62.
7.Tsalis K, Blouhos K, Vasiliadis K, Tsachalis T, Angelopoulos S, Betsis D. Sebaceous gland tumors and internal malignancy in the context of Muir-Torre syndrome. A case report and review of the literature. World J Surg Oncol. 2006 Feb 8;4:8.
8.PontiG,LosiL,PedroniM,Lucci-CordiscoE,DiGregorioC,Pellacani G,SeidenariS.ValueofMLH1andMSH2mutationsintheappearance
SGC and Muir–Torre’s Syndrome 505
of Muir-Torre syndrome phenotype in HNPCC patients presenting sebaceousglandtumorsorkeratoacanthomas.JInvestDermatol.2006 Oct;126(10):2302-7. Epub 2006 Jul 6.
9.Bertholom JL, Guyomard JL, Stock N, Dugast C, Martinel C, Chatel MA,CharlinJF.SebaceoustumorsoftheeyelidsinapatientwithMuirTorre syndrome J Fr Ophtalmol. 2006 Jun;29(6):654-8.
10.MarazzaG,MasouyéI,TaylorS,PrinsC,GaudinT,SauratJH,French LE. An illustrative case of Muir-Torre syndrome: contribution of immunohistochemical analysis in identifying indicator sebaceous lesions. Arch Dermatol. 2006 Aug;142(8):1039-42.
11.JonesB,OhC,MangoldE,EganCAMuir-Torresyndrome:Diagnostic and screening guidelines. Australas J Dermatol. 2006 Nov;47(4):266- 9.
12.Levi Z, Hazazi R, Kedar-Barnes I, Hodak E, Gal E, Mor E, Niv Y, WinklerJ.Switchingfromtacrolimustosirolimushaltstheappearance of new sebaceous neoplasms in Muir-Torre syndrome. Am J Transplant. 2007 Feb;7(2):476-9. Epub 2007 Jan 4.
13.Al-Shobaili HA, AlGhamdi KM, Al-Ghamdi WA. Cystic sebaceous carcinoma: is it a constant pathognomic marker for Muir-Torre syndrome? J Drugs Dermatol. 2007 May;6(5):540-3.
14.Lachiewicz AM, Wilkinson TM, Groben P, Ollila DW, Thomas NE. Muir-Torre syndrome. Am J Clin Dermatol. 2007;8(5):315-9.
15.YanabaK,NakagawaH,TakedaY,KoyamaN,SuganoK.Muir-Torre syndrome caused by partial duplication of MSH2 gene by Alumediated nonhomologous recombination. Br J Dermatol. 2008 Jan;158(1):150-6. Epub 2007 Oct 17.
16.Chhibber V, Dresser K, Mahalingam M. MSH-6: extending the reliabilityofimmunohistochemistryasascreeningtoolinMuir-Torre syndrome. Mod Pathol. 2008 Feb;21(2):159-64. Epub 2007 Dec 7.
17.Satarkar S, Munshi M, Kotwal M, Bobhate S. Muir Torre syndrome: a case report. Indian J Pathol Microbiol. 2007 Oct;50(4):804-5.
18.Ponti G, Ponz de Leon M. Muir-Torre syndrome. Lancet Oncol. 2005 Dec;6(12):980-7.
19.Song A, Carter KD, Syed NA, Song J, Nerad JA. Sebaceous cell carcinomaoftheocularadnexa:clinicalpresentations,histopathology, andoutcomes.OphthalPlastReconstrSurg.2008May-Jun;24(3):194- 200.
20.Ho VH, Ross MI, Prieto VG, Khaleeq A, Kim S, Esmaeli B. Sentinel lymph node biopsy for sebaceous cell carcinoma and melanoma of the ocular adnexa Arch Otolaryngol Head Neck Surg. 2007 Aug;133(8):820-6.
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21.Spencer JM, Nossa R, Tse DT, Sequeira M. Sebaceous carcinoma of the eyelid treated with Mohs micrographic surgery. J Am Acad Dermatol. 2001 Jun;44(6):1004-9.
22.Muqit MM, Roberts F, Lee WR, Kemp E. Improved survival rates in sebaceous carcinoma of the eyelid. Eye. 2004 Jan;18(1):49-53.
23.ChaoAN,ShieldsCL,KremaH,ShieldsJA.Outcomeofpatientswith periocularsebaceousglandcarcinomawithandwithoutconjunctival intraepithelial invasion. Ophthalmology. 2001 Oct;108(10):1877-83.
24.ShieldsCL,NaseripourM,ShieldsJA,EagleRCJr.Topicalmitomycin- C for pagetoid invasion of the conjunctiva by eyelid sebaceous gland carcinoma. Ophthalmology. 2002 Nov;109(11):2129-33.
25.Tumuluri K, Kourt G, Martin P. Mitomycin C in sebaceous gland carcinomawithpagetoidspread.BrJOphthalmol2004May;88(5):718- 9.
26.Murthy R, Honavar SG, Burman S, Vemuganti GK, Naik MN, Reddy VA. Neoadjuvant chemotherapy in the management of sebaceous gland carcinoma of the eyelid with regional lymph node metastasis. Ophthal Plast Reconstr Surg 2005 Jul;21(4):307-9.
27.Paschal BR, Bagley CS. Sebaceous gland carcinoma of the eyelid: completeresponsetosequentialcombinationchemotherapyNCMed J. 1985 Sep;46(9):473-4.
28.Stafanous SN. The switch flap in eyelid reconstruction .Orbit. 2007 Dec;26(4):255-62.
29.Henriquez A.S. Arruga A. Sebaceous Carcinoma Presenting as Dry eye Syndrome. Klinische Monatsblatter Fur Augenheilkunde 1979;175 (3):318-21.
30.Heyderman E. et al. Epithelial Markers in Primary Skin Cancer Histopathology 1984;8(3):423-34.
31.Harvey JT. Anderson RL. The Management of Meibomian Gland Carcinoma Ophthalmic surgery 1982;13(1):56-61.
32.26. Deregibus P. Battezzati G.Su due casi di carcinoma sebaceo Minerva Medica 1982;73(5):213-7.
33.21. Yen MT, et al. Radiation Therapy for Local Control of Eyelid Sebaceous Cell Carcinoma Ophthal Plast Reconstr Surg 2000 May, 16(3):211-5.
34.Rao NA, Hidayat AA, McLean IW, Zimmerman LE. Sebaceous Carcinoma Of Ocular Adnexa Human Pathology 1982;13(2):113-22.
INTRODUCTION
Medical science changes with time as more and more people submit their audits and personal experience to journals. These reports and studies make the understanding of diseases more clear. At times the natural history of diseases becomes clearer and at other times we realize that maybe we as humans respond differently with time. Sometimes historical mistakes are corrected but at times we might be committing newer mistakes. This chapter is an effort at looking at present updated literature on pediatric oculoplastic procedures in a critical manner with a view to applying the recent evidence to everyday clinical practice. The commonest pediatric oculolastic procedures are addressed here. Considering the unique problems presented by childhood oculoplastic conditions it seems appropriate to have pediatric oculoplasty as a separate speciality.
CONGENITAL DACRYOCYSTITIS UPDATE
The often quoted incidence of 6% was derived from a study of 200 consecutive live births in the 1940s. Actual incidences may vary from 1.2 to 30%, the disorder being commoner in children with craniofacial disorders and Down’s syndrome.
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Syndromes
Lacrimo-auriculo-dento-digital Syndrome (LADD)1
An autosomal dominant pattern of inheritance caused by FGF10 mutations with variable expressivity including cupshaped ears, deafness, unilateral choanal atresia, bilateral nasolacrimal duct obstruction, xerostomia, alacrima due to congenital absence of lacrimal glands, agenesis of salivary glands, chronic dacryocystitis, keratoconjunctivitis sicca, ptosis, nail dysplasia of the thumb, shortness of fifth toe, temporal bone abnormality and epilepsy. Renal and urogenital anomalies may be seen variably. In the author’s experience these findings do not always occur in these clusters but if one of these is seen in association with congenital dacryocystitis then the others should be looked for as they do require a multispecialty approach to management.
Microbiological Spectrum and Sensitivities
A recent study 2 conducted at Madurai, India concluded that gram-positive bacteria are the most frequent isolates with Streptococcus pneumoniae being the commonest. Among gramnegative bacteria the most frequent isolate was Hemophilus influenzae. Candida tropicalis has emerged as a new organism. Gram-positive bacteria are sensitive to chloramphenicol, vancomycin, and ofloxacin and gram-negative bacteria to ofloxacin and ciprofloxacin.
Treatment
Probing
Timing: In their series of 192 children with nasolacrimal duct obstruction out of 3950 children lipiec et al 3 reported that in majority of children(77%), the nasolacrimal duct obstruction