Orbitopatia basedowiana: percorso diagnostico-terapeutico · 2018-12-13 · Roma, 7-9 novembre 2014 Indicazioni alla radioterapia e chirurgia orbitaria Orbitopatia basedowiana: percorso

Roma, 7-9 novembre 2014

Indicazioni alla radioterapia e chirurgia orbitaria

Orbitopatia basedowiana: percorso diagnostico-terapeutico

Claudio Marcocci Dipartimento di Medicina Clinica e Sperimentale

Universita’ di Pisa U.O Endocrinologia 2

Azienda Ospedaliero-Universitaria di Pisa


Rationale for the use of orbital radiotherapy for GO

•  Nonspecific anti-inflammatory effect

•  Radiosensitivity of T-lymphocytes infiltrating the orbit

•  Reduction of glycosaminoglycan secretion


Protocol of orbital radiotherapy for GO

•  Supervoltage apparatus (linear accelerator, 4-6 MeV)

•  2 Gy per day in 10 daily doses over a 2-week period [cumulative dose, 20 Gy; lower cumulative doses (10 Gy) may be as effectiv]

•  Irradiation field: 4x4 cm lateral field, slightly angled posteriorly


Comparison of orbital radiotherapy and glucocorticoids for GO

0

3

6

9

12

15

18

Responders Nonresponders

Radiotherapy+placebo

Steroids+sham irradiation

N. o

f pat

ient

s

(Prummel, 1993)


Radiotherapy for Graves’ orbitopathy: randomized placebo-controlled study

Mourits MP et al, Lancet 2000

•  Treatment outcome was successful in about 60% of irradiated patients, compared 30% of sham irradiated patients at 24 weeks

•  This difference was mostly due to improvement of eye muscle motility

•  A more rapid fall of disease activity occurred in irradiated patients

•  No difference between the two groups in eyelid swelling and proptosis


Prummel et al. JCEM 2004

•  Improvement of eye muscle motility and diplopia •  Less need for further treatments


Oral glucocorticoids or radiotherapy alone vs combined therapy for GO

OI

(Bartalena, 1983, Marcocci, 1991)

p< 0.005 p< 0.01


Orbital irradiation combined with oral glucocorticoids

•  The combined regimen exploits the prompter effect of glucocorticoids and the more sustained action of irradiation

•  Orbital radiotherapy reduces the rate of recurrence observed with glucocorticoids alone, by shortening the active phase of the disease

•  Glucocorticoids prevent the transient exacerbations of eye manifestations caused by irradiation


Side effects and complications of orbital radiotherapy

Acute effects Skin irritation and temporal hair loss Transient exacerbation of inflammatory signs

Late effects Cataract Retinopathy Radiation-induced tumors


Radiation cataract

13/160

8.0%

LA

8/44

18.0 %

CU

p=0.1

21/204

10.2 % Total

Mean age 61 yr Mean latency 12 yr


Orbital radiotherapy and radiation cataract

Marcocci 2004


Orbital RT and secondary tumors

Medical history (n=204)

157 patients CT scan of the orbit and adjacent regions


Retinal changes vs retinopathy

median (range)

< 5 1.5 (1-5)

>5 23 (10-50)

n= 18 5 patients

9 4 2 1 2

micro’s 1 2 3 4 5

patients 1 1 1 1 1

micro’s 10 11 23§ 35§ 50§

Visual acuity 0.85 ± 0.2

0.66 ± 0.2*

* p<0.05

§ DM

Wakelkamp et al., Ophthalmology 2004


Management of moderate to severe GO: Orbital radiotherapy

! OR should be considered in patients with active GO who have diplopia or restricted motility (Ib, A)

!  Lower cumulative doses (10 Gy) may be as effective as and better tolerated than higher doses (20 Gy) (Ib, A). Doses >20 Gy are not recommended (IV, C)

! Caution should be exercised about administering OR to patients younger than 35 yr; OR must be avoided in patients with diabetic retinopathy and/or severe hypertension (III, B)

! Combination of oral GCs and OR is more effective than either treatment alone (Ib, A), but RCTs indicating that combination of iv GCs with OR is better than iv GCs alone are lacking (IV, C)


Orbital decompression: Rationale

Provides means for immediate expansion of orbital contents into a more capacious volume

– 0.67 ml in orbital volume = 1 mm reduction in proptosis

– N of orbital walls removed: 1 wall = 2-3 mm reductionin proptosis 4 walls = 14-16 mm reduction in

proptosis


Orbital decompression: Indications

Orbital decompression is a common treatment for Graves’ orbitopathy (GO)

Indications: •  Optic neuropathy (not responsive to high dose ivGC) •  Severe proptosis

•  Corneal exposure •  Subluxation/luxation of eye globes

•  Cosmetic/Rehabilitative purposes


Clin Endocrinol 2005


Clin Endocrinol 2005


Orbital decompression: Indications

Orbital decompression is a common treatment for Graves’ orbitopathy (GO)

Indications: •  Optic neuropathy (not responsive to high dose ivGC) •  Severe proptosis

•  Corneal exposure •  Subluxation/luxation of eye globes

•  Cosmetic/Rehabilitative purposes


•  Dural damage with cerebrospinal fluid leak and/or pneumocephalus

•  Iatrogenic damage of the optic nerve

•  Intraorbital haemorrhage

•  Periorbital hypoesthesia

•  Worsening of a pre-existent diplopia or de-novo development of diplopia

Complications


SQUINT SURGERY

PRE-OP.

2 months POST-OP.


Functional

EYELID SURGERY


Functional

Aesthetic

EYELID SURGERY


Management of moderate to severe GO:Surgery

!  The timing and order of surgical interventions should be carefully planned (IV, C)

! A fixed sequence of correction (if needed) has to be respected: orbital decompression, squint surgery, lid lengthening with or followed by blepharoplasty/browplasty (III, B)

! Rehabilitative decompression surgery should only be performed in euthyroid patients with GO quiescent for at least 6 months (III, B)

! Rehabilitative surgery should only be undertaken in centres with appropriate expertise (IV, C)


Documents

Orbitopatia basedowiana: percorso diagnostico-terapeutico · 2018-12-13 · Roma, 7-9 novembre 2014 Indicazioni alla radioterapia e chirurgia orbitaria Orbitopatia basedowiana: percorso